Set Analyses:
Advanced Search

Advanced Search

 
Search By
Section (entire)
for


 
or upload a file of gene symbols


Category   Symbol Source: HGNC EntrezGene Ensembl GeneCards RNA genes CroW21
GIFtS

GCDH Gene

protein-coding   GIFtS: 66
GCID: GC19P013001

Glutaryl-CoA Dehydrogenase

(Previous name: glutaryl-Coenzyme A dehydrogenase)
Alzheimer's & Parkinson's Diseases Congress
  See related diseases
at  

(According to 1HGNC, 2Entrez Gene,
3UniProtKB/Swiss-Prot, 4UniProtKB/TrEMBL, 5OMIM, 6GeneLoc, 7Ensembl, 8DME, 9miRBase, 10fRNAdb, 12H-InvDB, 13NCBI, 14NONCODE, and/or 15RNAdb)
About This Section

Aliases
Glutaryl-CoA Dehydrogenase1 2     Glutaryl-CoA Dehydrogenase, Mitochondrial2
Glutaryl-Coenzyme A Dehydrogenase1 2     EC 1.3.8.63
GCD2 3     EC 1.3.998
ACAD52     EC 1.3.99.78

External Ids:    HGNC: 41891   Entrez Gene: 26392   Ensembl: ENSG000001056077   OMIM: 6088015   UniProtKB: Q929473   

Export aliases for GCDH gene to outside databases

Previous GC identifers: GC19P013133 GC19P013224 GC19P012847 GC19P012862 GC19P012863 GC19P012573


(According to Entrez Gene, Tocris Bioscience, Wikipedia's Gene Wiki, PharmGKB,
UniProtKB/Swiss-Prot, and/or UniProtKB/TrEMBL)
About This Section

Entrez Gene summary for GCDH Gene:
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It catalyzes the oxidative
decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine,
L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor.
The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result
in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative
splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on
chromosome 12. (provided by RefSeq, Mar 2013)

GeneCards Summary for GCDH Gene: 
GCDH (glutaryl-CoA dehydrogenase) is a protein-coding gene. Diseases associated with GCDH include glutaric acidemia type i, and d-2-hydroxyglutaric aciduria, and among its related super-pathways are Tryptophan metabolism and Metabolic pathways. GO annotations related to this gene include fatty-acyl-CoA binding and flavin adenine dinucleotide binding. An important paralog of this gene is ACADL.

UniProtKB/Swiss-Prot: GCDH_HUMAN, Q92947
Function: Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative
pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its
electron acceptor. Isoform Short is inactive




(According to GeneLoc and/or HGNC, and/or
Entrez Gene (NCBI build 37),
and/or miRBase,
Genomic Views according to UCSC (hg19) and Ensembl (release 73), Regulatory elements and Epigenetics data according to QIAGEN, SABiosciences, and/or SwitchGear Genomics)
About This Section
RefSeq DNA sequence:
NC_000019.9  NC_018930.2  NT_011295.11  
Regulatory elements:
   SABiosciences Regulatory transcription factor binding sites in the GCDH gene promoter:
         AML1a   
         Other transcription factors

SwitchGear Promoter luciferase reporter plasmidGCDH promoter sequence
   Search SABiosciences Chromatin IP Primers for GCDH

Epigenetics:
QIAGEN PyroMark CpG Assay predesigned Pyrosequencing DNA Methylation assays in human, mouse, rat GCDH


Genomic Location:
Genomic View: UCSC Golden Path with GeneCards custom track

Entrez Gene cytogenetic band: 19p13.2   Ensembl cytogenetic band:  19p13.2   HGNC cytogenetic band: 19p13.2

GCDH Gene in genomic location: bands according to Ensembl, locations according to (and/or Entrez Gene and/or Ensembl if different)
GCDH gene location

GeneLoc information about chromosome 19         GeneLoc Exon Structure

GeneLoc location for GC19P013001:  view genomic region     (about GC identifiers)

Start:
13,001,840 bp from pter      End:
13,025,021 bp from pter
Size:
23,182 bases      Orientation:
plus strand

(According to UniProtKB, HORDE, neXtProt, Ensembl, and/or Reactome, Modification sites according to PhosphoSitePlus, Specific Peptides from DME, Protein expression images according to data from SPIRE 1MOPED, 2PaxDb, and 3MAXQB RefSeq according to NCBI, PDB rendering according to OCA and/or Proteopedia, Recombinant Proteins from EMD Millipore, R&D Systems, GenScript, Enzo Life Sciences, OriGene, Novus Biologicals, Sino Biological, ProSpec, and/or Cloud-Clone Corp.,
Biochemical Assays by EMD Millipore, R&D Systems, OriGene, GenScript, Cell Signaling Technology, Enzo Life Sciences, and/or Cloud-Clone Corp., Ontologies according to Gene Ontology Consortium 01 Oct 2013 and Entrez Gene, Antibodies by EMD Millipore, R&D Systems, GenScript, Cell Signaling Technology, OriGene, Novus Biologicals, Thermo Fisher Scientific, LSBio, Abcam, and/or Cloud-Clone Corp.)
About This Section

UniProtKB/Swiss-Prot: GCDH_HUMAN, Q92947 (See protein sequence)
Recommended Name: Glutaryl-CoA dehydrogenase, mitochondrial precursor  
Size: 438 amino acids; 48127 Da
Cofactor: FAD
Subunit: Homotetramer
Subcellular location: Mitochondrion matrix
4 PDB 3D structures from and Proteopedia for GCDH:
1SIQ (3D)        1SIR (3D)        2R0M (3D)        2R0N (3D)    
Secondary accessions: A8K2Z2 O14719
Alternative splicing: 2 isoforms:  Q92947-1   Q92947-2   

Explore the universe of human proteins at neXtProt for GCDH: NX_Q92947

Explore proteomics data for GCDH at MOPED 

Post-translational modifications:

  • View modification sites using PhosphoSitePlus
  • View neXtProt modification sites for NX_Q92947

  • 4/9 DME Specific Peptides for GCDH (Q92947) (see all 9)
     FGLTEPN  VWARCED  VNTYEGTHD  PIYAYGSEEQ 

    GCDH Protein expression data from MOPED1, PaxDb2 and MAXQB3 :    About this image 

    GCDH Protein Expression
    REFSEQ proteins (2 alternative transcripts): 
    NP_000150.1  NP_039663.1  

    ENSEMBL proteins: 
     ENSP00000222214   ENSP00000465618   ENSP00000466845   ENSP00000468125   ENSP00000468452  
     ENSP00000465770   ENSP00000468584   ENSP00000468625   ENSP00000467735   ENSP00000394872  
     ENSP00000394821  
    Reactome Protein details: Q92947
    Human Recombinant Protein Products for GCDH: 
    Browse Purified and Recombinant Proteins at EMD Millipore
    Browse R&D Systems for human recombinant proteins
    Browse recombinant and purified proteins available from Enzo Life Sciences
    OriGene Purified Protein for GCDH
    OriGene Protein Over-expression Lysate for GCDH
    OriGene MassSpec for GCDH 
    OriGene Custom Protein Services for GCDH
    GenScript Custom Purified and Recombinant Proteins Services for GCDH
    Novus Biologicals GCDH Proteins
    Novus Biologicals GCDH Lysates
    Browse Sino Biological Recombinant Proteins
    Browse Sino Biological Cell Lysates 
    ProSpec Recombinant Protein for GCDH
    Cloud-Clone Corp. Proteins for GCDH 

    Gene Ontology (GO): 3 cellular component terms (GO ID links to tree view):    About this table

    GO IDQualified GO termEvidencePubMed IDs
    GO:0005739mitochondrion TAS8541831
    GO:0005743mitochondrial inner membrane IEA--
    GO:0005759mitochondrial matrix TAS--

    GCDH for ontologies           About GeneDecksing



    GCDH Antibody Products: 
    Browse EMD Millipore's Extensive Line of Mono- and Polyclonal Antibodies
    Browse R&D Systems for Antibodies
    Browse OriGene Antibodies
    OriGene Custom Antibody Services for GCDH
    GenScript Custom Superior Antibodies Services for GCDH
    Novus Biologicals GCDH Antibodies
    Abcam antibodies for GCDH
    Cloud-Clone Corp. Antibodies for GCDH 
    ThermoFisher Antibodies for GCDH
    LSBio Antibodies in human, mouse, rat for GCDH 

    Assay Products for GCDH: 
    Browse Kits and Assays available from EMD Millipore
    OriGene Custom Assay Services for GCDH
    Browse R&D Systems for biochemical assays
    GenScript Custom Assay Services for GCDH
    Browse Enzo Life Sciences for kits & assays
    Cloud-Clone Corp. ELISAs for GCDH 
    Cloud-Clone Corp. CLIAs for GCDH


    (According to HGNC, IUPHAR, InterPro, ProtoNet, UniProtKB, and/or BLOCKS, Sets of similar genes according to GeneDecks)
    About This Section
    5/7 InterPro protein domains (see all 7):
     IPR006090 Acyl-CoA_Oxase/DH_1
     IPR013786 AcylCoA_DH/ox_N
     IPR006091 Acyl-CoA_Oxase/DH_cen-dom
     IPR006089 Acyl-CoA_DH_CS
     IPR009100 AcylCoA_DH/oxidase

    Graphical View of Domain Structure for InterPro Entry Q92947

    ProtoNet protein and cluster: Q92947

    1 Blocks protein domain: IPB006089 Acyl-CoA dehydrogenase

    UniProtKB/Swiss-Prot: GCDH_HUMAN, Q92947
    Similarity: Belongs to the acyl-CoA dehydrogenase family


    GCDH for domains           About GeneDecksing


    (According to 1UniProtKB, Genatlas, LifeMap Discovery™, IUBMB, and/or 2DME, Human phenotypes from GenomeRNAi, Animal models from MGI Mar 06 2013, inGenious Targeting Laboratory, genOway,
    bound targets from SABiosciences, miRNA Gene Targets from miRTarBase, shRNA from OriGene, RNAi from EMD Millipore, siRNAs from OriGene, QIAGEN, microRNA from QIAGEN, Gene Editing from DNA2.0, Sirion Biotech, Clones from EMD Millipore, OriGene, SwitchGear Genomics, GenScript, Sino Biological, DNA2.0, Vector BioLabs, and Sirion Biotech, Cell Lines from GenScript, LifeMap BioReagents, In Situ Hybridization Assays from Advanced Cell Diagnostics, Ontologies according to Gene Ontology Consortium 01 Oct 2013 via Entrez Gene.)
    About This Section

    Molecular Function:

         UniProtKB/Swiss-Prot Summary: GCDH_HUMAN, Q92947
    Function: Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative
    pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its
    electron acceptor. Isoform Short is inactive
    Catalytic activity: Glutaryl-CoA + electron-transfer flavoprotein = (E)-but-2-enoyl-CoA + CO(2) + reduced
    electron-transfer flavoprotein

         Genatlas biochemistry entry for GCDH:
    glutaryl-CoA dehydrogenase,flavoprotein dependent,mitochondrial matrix,lysine oxidation

         Enzyme Numbers (IUBMB): EC 1.3.99.72 EC 1.3.992 EC 1.3.8.61

         Gene Ontology (GO): 5/6 molecular function terms (GO ID links to tree view) (see all 6):    About this table

    GO IDQualified GO termEvidencePubMed IDs
    GO:0000062fatty-acyl-CoA binding IEA--
    GO:0003995acyl-CoA dehydrogenase activity ----
    GO:0004361glutaryl-CoA dehydrogenase activity EXP--
    GO:0016491oxidoreductase activity ----
    GO:0016627oxidoreductase activity, acting on the CH-CH group of donors ----
         
    GCDH for ontologies           About GeneDecksing


    Phenotypes:
         4 MGI mutant phenotypes (inferred from 1 allele(MGI details for Gcdh):
     behavior/neurological  homeostasis/metabolism  nervous system  renal/urinary system 

    GCDH for phenotypes           About GeneDecksing

    Animal Models:
       inGenious Targeting Laboratory - Custom generated mouse model solutions for GCDH 
       inGenious Targeting Laboratory - Custom generated inducible mouse model solutions for GCDH

       genOway customized KO model: permanent, tissue-specific or time-controlled inactivation for GCDH 
       genOway customized Knockin model: humanization, point mutation, expression monitoring, etc. for GCDH 

    miRNA
    Products:
        
    QIAGEN Custom miScript Target Protector blocks miRNA-binding site of human, mouse, rat GCDH
    2 QIAGEN miScript miRNA Assays for microRNAs that regulate GCDH:
    hsa-miR-124 hsa-miR-506
    SwitchGear 3'UTR luciferase reporter plasmidGCDH 3' UTR sequence
    Inhib. RNA
    Products:
        
    Browse for Gene Knock-down Tools from EMD Millipore
    OriGene RNAi products in human, mouse, rat for GCDH
    QIAGEN FlexiTube/FlexiPlate siRNA for gene silencing in human, mouse, rat GCDH

    Gene Editing
    Products:
    DNA2.0 Custom Protein Engineering Service for GCDH
    Sirion Biotech Customized adenovirus for overexpression of GCDH

    Clone
    Products:
         
    Browse Clones for the Expression of Recombinant Proteins Available from EMD Millipore
    OriGene clones in human, mouse for GCDH (see all 11)
    OriGene ORF clones in mouse, rat for GCDH
    OriGene custom cloning services - gene synthesis, subcloning, mutagenesis, variant library, vector shuttling
    GenScript: all cDNA clones in your preferred vector (see all 2): GCDH (NM_000159)
    Sino Biological Human cDNA Clone for GCDH
    DNA2.0 Custom Codon Optimized Gene Synthesis Service for GCDH
    Vector BioLabs ready-to-use adenovirus/AAV for human, mouse, rat GCDH
    Sirion Biotech Customized lentivirus for stable overexpression of GCDH 
                         Customized lentivirus expression plasmids for stable overexpression of GCDH 

    Cell Line
    Products:
         
    GenScript Custom overexpressing Cell Line Services for GCDH
    Search LifeMap BioReagents cell lines for GCDH
    In Situ Assay
    Products:
       

     
    Advanced Cell Diagnostics RNAscope RNA in situ hybridization assays for GCDH


    (Pathways according to EMD Millipore, R&D Systems, Cell Signaling Technology, KEGG, PharmGKB, BioSystems, Sino Biological, Reactome, Tocris Bioscience, GeneGo (Thomson Reuters), QIAGEN, and/or UniProtKB, Sets of similar genes according to GeneDecks, Interaction Networks according to SABiosciences, and/or STRING, Interactions according to 1UniProtKB, 2MINT, 3I2D, and/or 4STRING, with links to IntAct and Ensembl, Ontologies according to Gene Ontology Consortium 01 Oct 2013 via Entrez Gene).
    About This Section

    SuperPaths for GCDH About   (see all 8)                                                                                              See pathways by source

    SuperPathContained pathways About
    1Tryptophan metabolism
    Tryptophan metabolism0.46
    Tryptophan metabolism0.46
    2Metabolism
    Metabolism0.40
    Metabolic pathways0.40
    3Fatty Acid Beta Oxidation
    Fatty Acid Beta Oxidation0.35
    Fatty acid degradation0.32
    4tryptophan utilization II
    tryptophan utilization II0.70
    tryptophan utilization I0.70
    5tryptophan degradation to 2-amino-3-carboxymuconate semialdehyde
    tryptophan degradation III (eukaryotic)0.50
    glutaryl-CoA degradation0.40

    Pathways by source                                                                                                                                                                 See SuperPaths
    Show all pathways


    5/6 BioSystems Pathways for GCDH (see all 6)
        glutaryl-CoA degradation
    Fatty Acid Beta Oxidation
    Tryptophan metabolism
    tryptophan degradation III (eukaryotic)
    tryptophan utilization II


    3        Reactome Pathways for GCDH
        Lysine catabolism
    Metabolism
    Metabolism of amino acids and derivatives


    4         Kegg Pathways  (Kegg details for GCDH):
        Fatty acid degradation
    Lysine degradation
    Tryptophan metabolism
    Metabolic pathways

    UniProtKB/Swiss-Prot: GCDH_HUMAN, Q92947
    Pathway: Amino-acid metabolism; lysine degradation
    Pathway: Amino-acid metabolism; tryptophan metabolism


    GCDH for pathways           About GeneDecksing

    Interactions:

        Search SABiosciences Gene Network CentralTM Interacting Genes and Proteins Networks for GCDH

    STRING Interaction Network Preview (showing 5 interactants - click image to see 16)

    5/32 Interacting proteins for GCDH (Q929471, 3 ENSP000002222144) via UniProtKB, MINT, STRING, and/or I2D (see all 32)

    InteractantInteraction Details
    GeneCardExternal ID(s)
    NOS3P294741, 3EBI-1236978,EBI-1391623 I2D: score=1 
    PSEN1P497681, 3EBI-1236978,EBI-297277 I2D: score=1 
    GRB2P629931, 3, ENSP000003390074EBI-1236978,EBI-401755 I2D: score=1 STRING: ENSP00000339007
    MYCP011063, ENSP000003672074I2D: score=3 STRING: ENSP00000367207
    ECSITQ9BQ953, ENSP000002705174I2D: score=1 STRING: ENSP00000270517
    About this table

    Gene Ontology (GO): 5/9 biological process terms (GO ID links to tree view) (see all 9):    About this table

    GO IDQualified GO termEvidencePubMed IDs
    GO:0006554lysine catabolic process TAS--
    GO:0006568tryptophan metabolic process IEA--
    GO:0006637acyl-CoA metabolic process ----
    GO:0008152metabolic process ----
    GO:0019395fatty acid oxidation IEA--

    GCDH for ontologies           About GeneDecksing



    (Chemical Compounds according to UniProtKB, Enzo Life Sciences, EMD Millipore, Tocris Bioscience HMDB, BitterDB, and/or Novoseek, Ligands according to IUPHAR, and Drugs according to DrugBank, Enzo Life Sciences, and/or PharmGKB, with drugs/clinical trials/news search links to CenterWatch)
    About This Section

    GCDH for compounds           About GeneDecksing

    Browse Small Molecules at EMD Millipore
    Browse drugs & compounds from Enzo Life Sciences

    Browse Tocris compounds for GCDH

    10/36 HMDB Compounds for GCDH (see all 36)    About this table
    CompoundSynonyms CAS #PubMed Ids
    (2E)-Decenoyl-CoA(E)-S-2-decenoate Coenzyme A (see all 15)10018-95-8--
    (2E)-Dodecenoyl-CoA2-trans-Dodecenoyl-Coenzyme A (see all 4)1066-12-2--
    (2E)-Hexadecenoyl-CoAtrans-2-Hexadecenoyl-CoA (see all 4)4460-95-1--
    (2E)-Octenoyl-CoA(E)-S-2-octenoate Coenzyme A (see all 16)10018-94-7--
    (2E)-Tetradecenoyl-CoA(2E)-Tetradecenoyl-Coenzyme A (see all 8)38795-33-4--
    2-Methylbutyryl-CoA2-methylbutanoyl-CoA (see all 11)6712-02-3--
    2-Methylhexanoyl-CoA2-Methylhexanoyl-CoEnzyme A (see all 5)----
    3Z-dodecenoyl-CoAS-[2-[3-[[(2R)-4-[[[(2R,3R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-phosphonooxyoxolan-2-yl]methoxy-hydroxyphosphoryl]oxy-hydroxyphosphoryl]oxy-2-hydroxy-3,3-dimethylbutanoyl]amino]propanoylamino]ethyl] (Z)-dodec-3-enethioate (see all 4)2152-91-2--
    Acetyl-CoAS-Acetyl coenzyme A (see all 13)72-89-9--
    Acrylyl-CoAAcryloyl coenzyme A (see all 14)5776-58-9--

    2 DrugBank Compounds for GCDH    About this table
    CompoundSynonyms CAS #TypeActionsPubMed Ids
    Flavin-Adenine Dinucleotide-- 146-14-5target--17139284 17016423
    S-4-Nitrobutyryl-Coa-- --target--17139284 17016423

    10 Novoseek inferred chemical compound relationships for GCDH gene    About this table
    Compound   -log (P-Val)   Hits   PubMed IDs for Articles with Shared Sentences (# sentences)
    3-hydroxyglutaric acid 97.8 3 14598231 (1), 17356845 (1), 18775954 (1)
    glutaric acid 95.4 10 11854167 (1), 14598231 (1), 17356845 (1), 15505389 (1) (see all 7)
    glutarylcarnitine 95.3 1 14598231 (1)
    glutaconic acid 92.2 1 15505389 (1)
    glutaryl-coa 90.2 2 12473778 (1), 16377226 (1)
    hydroxylysine 81.5 6 17879145 (1), 16466958 (1), 16641220 (1), 9600243 (1) (see all 5)
    carnitine 68.7 1 17957492 (1)
    acyl-coa 58.8 5 17176108 (1), 10960496 (1)
    lysine 49.6 6 16446282 (1), 16466958 (1), 9633984 (1), 9600243 (1) (see all 5)
    riboflavin 43 2 16377226 (2)

    Search CenterWatch for drugs/clinical trials and news about GCDH

    (Secondary structures according to fRNAdb,
    GenBank/EMBL/DDBJ Accessions according to
    Unigene (Build 236 Homo sapiens; Apr 25 2013) or GenBank,
    RefSeq according to Entrez Gene,
    DOTS (version 10), and/or AceView, transcript ids from Ensembl with links to UCSC,
    Conferences by KenesGroup, exon structure from GeneLoc, alternative splicing isoforms according to ASD and/or ECgene,
    RNAi Products from EMD Millipore,
    siRNAs from OriGene, QIAGEN, shRNA from OriGene, microRNA from QIAGEN,
    Tagged/untagged cDNA clones from OriGene, SwitchGear Genomics, GenScript, DNA2.0, Vector BioLabs, Sirion Biotech, Primers from OriGene, SABiosciences, and/or QIAGEN )
    About This Section

    REFSEQ mRNAs for GCDH gene (2 alternative transcripts): 
    NM_000159.3  NM_013976.3  

    Unigene Cluster for GCDH:

    Glutaryl-CoA dehydrogenase
    Hs.532699  [show with all ESTs]
    Unigene Representative Sequence: AK296446
    18 Ensembl transcripts including schematic representations, and UCSC links where relevant:
    ENST00000222214(uc002mvq.3 uc010xmt.2) ENST00000589039 ENST00000421816(uc010xms.2)
    ENST00000591470 ENST00000590445 ENST00000590530 ENST00000588905 ENST00000587072
    ENST00000590627 ENST00000587832 ENST00000585420 ENST00000585760 ENST00000591043
    ENST00000590472 ENST00000591050 ENST00000588242 ENST00000457854(uc002mvp.3)
    ENST00000422947(uc010xmu.2)

    miRNA
    Products:
         
    QIAGEN Custom miScript Target Protector blocks miRNA-binding site of human, mouse, rat GCDH
    2 QIAGEN miScript miRNA Assays for microRNAs that regulate GCDH:
    hsa-miR-124 hsa-miR-506
    SwitchGear 3'UTR luciferase reporter plasmidGCDH 3' UTR sequence
    Inhib. RNA
    Products:
         
    Browse for Gene Knock-down Tools from EMD Millipore
    OriGene RNAi products in human, mouse, rat for GCDH
    QIAGEN FlexiTube/FlexiPlate siRNA for gene silencing in human, mouse, rat GCDH
    Clone
    Products:
         
    OriGene clones in human, mouse for GCDH (see all 11)
    OriGene ORF clones in mouse, rat for GCDH
    OriGene custom cloning services - gene synthesis, subcloning, mutagenesis, variant library, vector shuttling
    GenScript: all cDNA clones in your preferred vector (see all 2): GCDH (NM_000159)
    DNA2.0 Custom Codon Optimized Gene Synthesis Service for GCDH
    Vector BioLabs ready-to-use adenovirus/AAV for human, mouse, rat GCDH
    Sirion Biotech Customized lentivirus for stable overexpression of GCDH 
                         Customized lentivirus expression plasmids for stable overexpression of GCDH 
    Primer
    Products:
        
    OriGene qPCR primer pairs and template standards for GCDH
    OriGene qSTAR qPCR primer pairs in human, mouse for GCDH
    SABiosciences RT2 qPCR Primer Assay in human, mouse, rat GCDH
      QIAGEN QuantiTect SYBR Green Assays in human, mouse, rat GCDH
      QIAGEN QuantiFast Probe-based Assays in human, mouse, rat GCDH

    Additional mRNA sequence: 

    AK098370.1 AK290407.1 AK296446.1 AK298772.1 AK300841.1 AK309892.1 BC002579.2 BT006706.1 
    U69141.1 

    14 DOTS entries:

    DT.415866  DT.100773302  DT.121410645  DT.121410639  DT.95088952  DT.100757337  DT.40132113  DT.100700204 
    DT.100829507  DT.121410591  DT.92434797  DT.95088957  DT.100843711  DT.99959158 

    24/225 AceView cDNA sequences (see all 225):

    BG392199 CR599782 AA322355 CR600877 CR591108 BE254722 CR618805 CR593626 
    NM_013976 BX090910 BI755368 CR626122 CA432442 CB114901 BM830975 BQ068107 
    BM823186 NM_000159 AU139663 BM831180 CR622862 BF062558 CR621071 CR615192 

    GeneLoc Exon Structure


    (RNA expression data according to H-InvDB, NONCODE, miRBase, and RNAdb, Expression images according to data from BioGPS, Illumina Human BodyMap, and CGAP SAGE, Sets of similar genes according to GeneDecks, in vivo and in vitro expression data from LifeMap Discovery™, plus additional links to Genevestigator, and/or SOURCE, and/or BioGPS, and/or UniProtKB,
    PCR Arrays from SABiosciences, Primers from OriGene, SABiosciences, and/or QIAGEN, In Situ Hybridization Assays from Advanced Cell Diagnostics)
    About This Section

    GCDH expression in normal human tissues (normalized intensities)      GCDH embryonic expression: see
    See probesets specificity/sensitivity at GeneAnnot
    About this imageBioGPS <intensity>2/3
    CGAP TAG: GAAGCAACTC
    GCDH Expression
    About this image


    GCDH expression in embryonic tissues and stem cells    About this table
    Data from LifeMap, the Embryonic Development and Stem Cells Database 
     5/31 selected tissues (see all 31) fully expand
     
     Colon (Gastrointestinal Tract)    fully expand to see all 3 entries
             colon ; peripheral nerve/ganglion   
     
     Tonsil (Hematopoietic System)    fully expand to see all 3 entries
             tonsil ; squamous epithelial cells   
     
     Epithelium (Uncategorized)    fully expand to see all 3 entries
             oral mucosa ; squamous epithelial cells   
     
     Testis (Reproductive System)    fully expand to see all 2 entries
             Leydig Cells Testis Interstitium
             seminal vesicle ; glandular cells   
     
     Pancreas (Endocrine System)    fully expand to see all 2 entries
             Islets of Langerhans
             pancreas ; exocrine glandular cells   

    See GCDH Protein Expression from SPIRE MOPED and PaxDB
    Genevestigator expression for GCDH

    SOURCE GeneReport for Unigene cluster: Hs.532699

    UniProtKB/Swiss-Prot: GCDH_HUMAN, Q92947
    Tissue specificity: Isoform 1 and isoform 2 are expressed in fibroblasts and liver

        SABiosciences Expression via Pathway-Focused PCR Arrays including GCDH: 
              Fatty Acid Metabolism in human mouse rat
              Amino Acid Metabolism II in human mouse rat
              Amino Acid Metabolism I in human mouse rat
              Molecular Toxicology PathwayFinder 384HT in human mouse rat

    Primer
    Products:
    OriGene qPCR primer pairs and template standards for GCDH
    OriGene qSTAR qPCR primer pairs in human, mouse for GCDH
    SABiosciences RT2 qPCR Primer Assay in human, mouse, rat GCDH
    QIAGEN QuantiTect SYBR Green Assays in human, mouse, rat GCDH
    QIAGEN QuantiFast Probe-based Assays in human, mouse, rat GCDH
    In Situ
    Assay Products:
     

     
    Advanced Cell Diagnostics RNAscope RNA in situ hybridization assays for GCDH

    (Orthologs according to 1,2HomoloGene (2older version, for species not in 1newer version), 3euGenes, 4SGD , 5MGI Mar 06 2013, with possible further links to Flybase and/or WormBase, and/or 6Ensembl pan taxonomic compara , Gene Trees according to Ensembl and TreeFam)
    About This Section

    This gene was present in the common ancestor of animals.

    Orthologs for GCDH gene from 7/19 species (see all 19)    About this table
    Organism Taxonomic
    classification
    Gene Description Human
    Similarity
    Orthology
    Type
    Details
    mouse
    (Mus musculus)
    Mammalia Gcdh1 , 5 glutaryl-Coenzyme A dehydrogenase1, 5 83.52(n)1
    87.59(a)1
      8 (41.28 cM)5
    2700761  NM_001044744.11  NP_001038209.21 
     848863935 
    chicken
    (Gallus gallus)
    Aves LOC7710981 glutaryl-CoA dehydrogenase, mitochondrial-like 79.06(n)
    82.3(a)
      771098  XM_001231725.2  XP_001231726.2 
    lizard
    (Anolis carolinensis)
    Reptilia GCDH6
    Uncharacterized protein
    73(a)
    1 ↔ 1
    2(27705187-27723446)
    African clawed frog
    (Xenopus laevis)
    Amphibia Xl.67132 Xenopus laevis transcribed sequence with weak similarity more 74.17(n)    BX852732.1 
    zebrafish
    (Danio rerio)
    Actinopterygii zgc777042 hypothetical protein MGC77704 77.79(n)   393860  BC063972.1 
    fruit fly
    (Drosophila melanogaster)
    Insecta CG95471 , 3 glutaryl-CoA dehydrogenase3
    CG95471
    68(a)3
    65.33(n)1
    69.25(a)1
      26D93
    339111  NM_135196.21  NP_609040.11 
    worm
    (Caenorhabditis elegans)
    Secernentea F54D5.73
    CELE_F54D5.71
    acyl-CoA dehydrogenase3
    Protein F54D5.71
    64(a)3
    61.39(n)1
    64.42(a)1
      II(11547428-11549088)3
    1747681  NM_064068.41  NP_496469.11 


    ENSEMBL Gene Tree for GCDH (if available)
    TreeFam Gene Tree for GCDH (if available)

    (Paralogs according to 1HomoloGene,
    2Ensembl, and 3SIMAP, Pseudogenes according to 4Pseudogene.org Build 68)
    About This Section
    Paralogs for GCDH gene
    ACADL2  ACAD82  ACADSB2  ACAD112  ACADS2  ACAD92  ACADM2  ACAD102  
    IVD2  ACADVL2  
    18/60 SIMAP similar genes for GCDH using alignment to 11 protein entries:     GCDH_HUMAN (see all proteins) (see all similar genes):
    WARS    APOPT1    SH2B3    SNRPF    TUBA1C    C12orf76
    CHID1    DCTN5    pp12301    LYRM4    MST132    ZNF547
    RBP7    FLJ00047    NF2    COQ5    ZNF564    LINC00596

    GCDH for paralogs           About GeneDecksing


    1 Pseudogenes.org Pseudogene for GCDH
    PGOHUM00000258685


    (SNPs/Variants according to the 1NCBI SNP Database, 2Ensembl, 3PupaSUITE, 4UniProtKB, and DNA2.0, Linkage Disequilibrium by HapMap, Structural Variations(CNVs/InDels/Inversions) from the Database of Genomic Variants, Mutations from the Human Gene Mutation Database (HGMD) and the Locus Specific Mutation Databases (LSDB), Blood group antigen gene mutations by BGMUT, Resequencing Primers from QIAGEN, Cancer Mutation PCR Arrays and Assays and Copy Number PCR Arrays from SABiosciences)
    About This Section

    10/320 SNPs in GCDH are shown (see all 320)    About this table     
    Genomic DataTranscription Related DataAllele Frequencies
    SNP IDValidClinical
    significance
    Chr 19 posSequence#AA
    Chg
    TypeMore#Allele
    freq
    PopTotal
    sample
    More
    ----------
    VAR_0003674
    Glutaric aciduria 1 (GA1)4--see VAR_0003672 R L mis40--------
    VAR_0003944
    Glutaric aciduria 1 (GA1)4--see VAR_0003942 L W mis40--------
    VAR_0004204
    Glutaric aciduria 1 (GA1)4--see VAR_0004202 A V mis40--------
    VAR_0004134
    Glutaric aciduria 1 (GA1)4--see VAR_0004132 R Q mis40--------
    VAR_0004024
    Glutaric aciduria 1 (GA1)4--see VAR_0004022 E K mis40--------
    VAR_0003894
    Glutaric aciduria 1 (GA1)4--see VAR_0003892 Y H mis40--------
    VAR_0004144
    Glutaric aciduria 1 (GA1)4--see VAR_0004142 H R mis40--------
    VAR_0003954
    Glutaric aciduria 1 (GA1)4--see VAR_0003952 R W mis40--------
    VAR_0004154
    Glutaric aciduria 1 (GA1)4--see VAR_0004152 N K mis40--------
    VAR_0003714
    Glutaric aciduria 1 (GA1)4--see VAR_0003712 R G mis40--------

    HapMap Linkage Disequilibrium report for GCDH (13001840 - 13025021 bp)

    Structural Variations
         Database of Genomic Variants (DGV) 3 variations for GCDH:    About this table     
    Variant IDTypeSubtypePubMed ID
    nsv911082CNV Loss21882294
    nsv833754CNV Loss17160897
    dgv3733n71CNV Loss21882294


    Human Gene Mutation Database (HGMD): GCDH
    SABiosciences Cancer Mutation PCR Assays
    SeqTarget long-range PCR primers for resequencing GCDH
    DNA2.0 Custom Variant and Variant Library Synthesis for GCDH

    (in which this Gene is Involved, According to MalaCards, OMIM, UniProtKB, the University of Copenhagen DISEASES database, Conferences by KenesGroup, Novoseek, Genatlas, GeneTests, GAD, HuGE Navigator, and/or TGDB.)
    About This Section
    OMIM gene information: 608801   
    OMIM disorders: 231670  
    UniProtKB/Swiss-Prot: GCDH_HUMAN, Q92947
  • Glutaric aciduria 1 (GA1) [MIM:231670]: An autosomal recessive metabolic disorder characterized by
    progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia. Note=The disease is
    caused by mutations affecting the gene represented in this entry

  • 18 diseases for GCDH:    About MalaCards
    glutaric acidemia type i    d-2-hydroxyglutaric aciduria    the organic acidemias: an    l-2-hydroxyglutaric aciduria
    corneal granular dystrophy    athetosis    2-hydroxyglutaric aciduria    neurologic diseases
    organic acidemia    breast cyst    cerebral palsy    corneal dystrophy
    metabolic disorders    cerebritis    tuberculosis    colorectal cancer
    alzheimer's disease    neuronitis

    5 diseases from the University of Copenhagen DISEASES database for GCDH:
    Corneal dystrophy     Organic acidemia     Cerebral palsy     Movement disease
    L-2-hydroxyglutaric aciduria

    GCDH for disorders           About GeneDecksing


    Congresses - knowledge worth sharing:  
    Alzheimer's & Parkinson's Diseases Congress (ADPD) 18 - 22 March 2015

    6 Novoseek inferred disease relationships for GCDH gene    About this table

    Disease   -log (P-Val)   Hits   PubMed IDs for Articles with Shared Sentences (# sentences)
    encephalopathy acute 74.7 1 11174631 (1)
    encephalopathy 34.8 1 1956737 (1)
    neurological disorders 32.4 3 17957492 (2), 15505395 (1)
    neurodegeneration 31.2 3 10960496 (1), 10649503 (1), 15318278 (1)
    cell damage 9.75 1 15505386 (1)
    neurodegenerative diseases 0.722 1 7490088 (1)

    Genetic Association Database (GAD): GCDH
    Human Genome Epidemiology (HuGE) Navigator: GCDH (4 documents)

    Export disorders for GCDH gene to outside databases

    (in PubMed. Associations of this gene to articles via 1Entrez Gene, 2UniProtKB/Swiss-Prot, 3HGNC, 4GAD, 5PharmGKB, 6HMDB, 7DrugBank, 8UniProtKB/TrEMBL, 9 Novoseek, and/or 10fRNAdb)
    About This Section

    PubMed articles for GCDH gene, integrated from 9 sources (see all 105):
    (articles sorted by number of sources associating them with GCDH)
        Utopia: connect your pdf to the dynamic
    world of online information

    1. Pork and human cDNAs encoding glutaryl-CoA dehydrogenase. (PubMed id 1438360)1, 2, 3 Goodman S.I.... Frerman F.E. (1992)
    2. Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH). (PubMed id 18775954)1, 2, 9 Keyser B.... Braulke T. (2008)
    3. Gene structure and mutations of glutaryl-coenzyme A dehydrogenase: impaired association of enzyme subunits that is due to an A421V substitution causes glutaric acidemia type I in the Amish. (PubMed id 8900227)1, 2, 9 Biery B.J.... Goodman S.I. (1996)
    4. Crystal structures of human glutaryl-CoA dehydrogenase with and without an alternate substrate: structural bases of dehydrogenation and decarboxylation reactions. (PubMed id 15274622)1, 2, 9 Fu Z.... Kim J.-J.P. (2004)
    5. The human glutaryl-CoA dehydrogenase gene: report of intronic sequences and of 13 novel mutations causing glutaric aciduria type I. (PubMed id 9600243)1, 2, 9 Schwartz M.... Brandt N.J. (1998)
    6. Glutaryl-CoA dehydrogenase mutations in glutaric acidemia (type I): review and report of thirty novel mutations. (PubMed id 9711871)1, 2, 9 Goodman S.I.... Elpeleg O.N. (1998)
    7. Assignment of human glutaryl-CoA dehydrogenase gene (GCDH) to the short arm of chromosome 19 (19p13.2) by in situ hybridization and somatic cell hybrid analysis. (PubMed id 8088809)1, 3, 9 Greenberg C.R....Goodman S.I. (1994)
    8. Genetic variants in nuclear-encoded mitochondrial gen es influence AIDS progression. (PubMed id 20877624)1, 4 Hendrickson S.L....O'Brien S.J. (2010)
    9. Variation at the NFATC2 locus increases the risk of t hiazolidinedione-induced edema in the Diabetes REduction Assessment with ramipr il and rosiglitazone Medication (DREAM) study. (PubMed id 20628086)1, 4 Bailey S.D....Anand S. (2010)
    10. Multiple loci influence erythrocyte phenotypes in the CHARGE Consortium. (PubMed id 19862010)1, 4 Ganesh S.K....Lin J.P. (2009)

    (in PubMed, OMIM, and NCBI Bookshelf)
    About This Section
     ANDOR
    Aliases
    Disorders
    Free Text  

      Query String
    PubMed
    OMIM
    NCBI Bookshelf
      (Note: In FireFox, select the above section and copy using Ctrl-C)

    (According to Entrez Gene, HGNC, AceView, euGenes, Ensembl, miRBase, ECgene, Kegg, and/or H-InvDB)
    About This Section
    Entrez Gene: 2639 HGNC: 4189 AceView: GCDH Ensembl:ENSG00000105607 euGenes: HUgn2639
    ECgene: GCDH Kegg: 2639 H-InvDB: GCDH

    (According to HUGE)
    About This Section
      --

    (According to PharmGKB, ATLAS, HORDE, IMGT, LEIDEN, UniProtKB/Swiss-Prot, and/or UniProtKB/TrEMBL,
    Wikipedia and/or GeneReviews via UniProtKB/Swiss-Prot)
    About This Section
    NameDescription
    PharmGKB entry for GCDH Pharmacogenomics, SNPs, Pathways
    GeneReviewshttp://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/GCDH

    (Patent information from GeneIP,
    Licensable technologies from WIS Yeda, Salk, Tufts,
    IP news from LifeMap Sciences, Inc.)
    About This Section
    Patent Information for GCDH gene:
    Search GeneIP for patents involving GCDH

    GeneCards and IP:
    Japan Patent Office Licenses GeneCards     European Patent Office Licenses GeneCards     Improving the IP Search



    (Antibodies, recombinant proteins, and assays from EMD Millipore, R&D Systems, OriGene, QIAGEN, GenScript, Cell Signaling Technology, SABiosciences, Novus Biologicals, Sino Biological, Enzo Life Sciences, Abcam, ProSpec, Cloud-Clone Corp., Thermo Fisher Scientific, LSBio, Gene Editing from DNA2.0 and Sirion Biotech, Clones from EMD Millipore, OriGene, GenScript, Sino Biological, DNA2.0, SwitchGear Genomics, Vector BioLabs, Sirion Biotech, Cell lines from GenScript, and LifeMap BioReagents, PCR Arrays from SABiosciences, Drugs and/or compounds from EMD Millipore, Tocris Bioscience, and/or Enzo Life Sciences, In Situ Hybridization Assays from
    Advanced Cell Diagnostics, Animal models from inGenious Targeting Laboratory, genOway)
    About This Section

     
     EMD Millipore Custom Antibody & Bulk Services
     EMD Millipore Preclinical / Clinical Development Services
     EMD Millipore Immunoassay Services
     EMD Millipore Target Screening & Profiling Services

      
     Browse Antibodies   Browse Cell Culture Products  
     Browse ELISAs   Browse Flow Cytometry Kits  
     Browse Primer Pairs   Browse Kinase Activity Assays/Reagents  
     Browse ELISpot Kits/Development Modules   Browse TFB/Immunoprecipitation Assays  
     Browse Apoptosis Detection Kits/Reagents   Browse Ubiquitin Proteasome Pathway (UPP) Assay Kits/Reagents  
     Browse DNA Damage/Repair Kits/Reagents   Browse Multiplex/Array Assay Kits/Reagents  
     Browse Cell Selection/Detection Kits/Reagents   Browse Secondary Antibodies/Controls/Staining Reagents  
     Browse Protease Activity Assays and Reagents   Browse Recombinant/Natural Proteins  
     Browse Stem Cell Products   Browse Tocris Biochemicals & Compounds  
     Browse cDNA Clones   Browse Proteome Profiler Antibody Arrays  
     Browse OriGene Antibodies   OriGene RNAi products in human, mouse, rat for GCDH  
     OriGene qPCR primer pairs and template standards for GCDH   OriGene Protein Over-expression Lysate for GCDH  
     OriGene MassSpec something-or-other for GCDH   OriGene clones in human, mouse for GCDH  
     OriGene qSTAR qPCR primer pairs in human, mouse for GCDH   OriGene Purified Protein for GCDH  
     OriGene ORF clones in mouse, rat for GCDH   OriGene custom cloning services - gene synthesis, subcloning, mutagenesis, variant library, vector shuttling  
     OriGene Custom Antibody Services for GCDH   OriGene Custom Protein Services for GCDH  

     
     
     QIAGEN Custom miScript Target Protector blocks miRNA-binding site of in human, mouse, rat GCDH
     QIAGEN SeqTarget long-range PCR primers for resequencing GCDH
     QIAGEN PyroMark CpG Assay predesigned Pyrosequencing DNA Methylation assays in human, mouse, rat GCDH
     QIAGEN FlexiTube/FlexiPlate siRNA for gene silencing in human, mouse, rat GCDH
     QIAGEN QuantiFast Probe-based Assays in human, mouse, rat GCDH
     QIAGEN QuantiTect SYBR Green Assays in human, mouse, rat GCDH
     GenScript Custom Purified and Recombinant Proteins Services for GCDH GenScript cDNA clones with any tag delivered in your preferred vector for GCDH
     GenScript Custom Assay Services for GCDH GenScript Custom Superior Antibodies Services for GCDH
     GenScript Custom overexpressing Cell Line Services for GCDH CloneReady with Over 120,000 Genes
     Gene Synthesis: Any Gene in Any Vector Vector-based siRNA and miRNA, Ready for Transfection
     Gene Mutant Library, Variants up to 10^11 Plasmid Preparation
     Custom Peptide Services
     Search for Antibodies & Assays

     Regulatory tfbs in GCDH promoter
     Search Chromatin IP Primers for GCDH
     RT2 qPCR Primer Assay in human, mouse, rat GCDH
     Search GNC Networks for GCDH
     SABiosciences PCR Arrays including human, mouse, rat GCDH
     Search Tocris compounds for GCDH
     Browse Sino Biological Proteins and Antibodies
     Browse Sino Biological Cell Lysates
     cDNA Clones for GCDH
     4000+ Proteins
     Search Sino Biological for antibodies, proteins & pathways
     Protein Production Services
     Transfection Reagents
     Protein A/G/L resins
     Isotyping reagents
     Search www.enzolifesciences.com for proteins, assays, substrates, inhibitors & antibodies
     Novus Tissue Slides
     GCDH antibodies
     GCDH proteins
     GCDH lysates
     Antibodies for GCDH
     See all of Abcam's Antibodies, Kits and Proteins for GCDH
     Custom Antibody / Protein Production Service
     Bulk Purchasing
     Advantages of Rabbit Monoclonal antibodies
     Abcam protocols and scientific support
     Recombinant Protein for GCDH




     Proteins for GCDH
     Antibodies for GCDH
     ELISAs for GCDH
     CLIAs for GCDH
     Search LifeMap BioReagents cell lines for GCDH
     Gene Synthesis
     Protein Engineering
     Variant Library Synthesis
     Codon Optimization
     Protein Production and Purification
     Advanced Cell Diagnostics RNAscope RNA in situ hybridization assays for GCDH
     SwitchGear 3'UTR luciferase reporter plasmids for GCDH
     SwitchGear Promoter luciferase reporter plasmids for GCDH
     ThermoFisher Antibodies for GCDH
     Vector BioLabs ready-to-use adenovirus/AAV for human, mouse, rat GCDH
     inGenious Targeting Laboratory - Custom generated mouse model solutions for GCDH
     inGenious Targeting Laboratory - Custom generated inducible mouse model solutions for GCDH
    Customized:
     lentivirus for stable overexpression of GCDH
     lentivirus expression plasmids for stable overexpression of GCDH
     adenovirus for overexpression of GCDH
     LSBio Antibodies in human, mouse, rat for GCDH
    Customized transgenic rodents for:
     Humanization
     Biomarker expression
     Off-target effect monitoring
     Translational medicine
     Tissue-specific gene expresssion
     Time-controlled gene expresssion
           
    GeneCards Homepage - Last full update: 23 Oct 2013 - Incrementals: 4 Nov 2013 , 7 Nov 2013 , 23 Jan 2014

    View Random Gene

    Category
    (GIFtS: 73)
    transforming growth factor, beta 1
    GIFtS Group
    The GeneCards human gene database gene index: 1 3 5 6 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z 


    Developed at the Crown Human Genome Center, Department of Molecular Genetics, the Weizmann Institute of Science

    Hot genes      Disease genes      GCDH gene at Home site.
    hostname: 356980-web2.xennexinc.com index build: 106 solr: 1.4