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Aliases & Descriptions for OGDH
(According to
1 HGNC ,
2 Entrez Gene ,
3 UniProtKB/Swiss-Prot ,
4 UniProtKB/TrEMBL , 5 OMIM , 6 GeneLoc
, and/or 7 Ensembl ,
8 miRBase )About This Section
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Aliases AKGDH 2 E1k 1 , 2 EC 1.2.4.2 3 H_DJ0691F11_gi16307008.sp_cds.1 2 OGDC 2
Descriptions 2-oxoglutarate dehydrogenase 2 Alpha-ketoglutarate dehydrogenase 3 oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) 2 oxoglutarate decarboxylase' 2 oxoglutarate dehydrogenase (lipoamide) 2 oxoglutarate dehydrogenase (succinyl-transferring) 2
Search outside databases for aliases for OGDH genePrevious GC identifers: GC07P044292 GC07P044387 GC07P044419
Summaries for OGDH (According to Entrez Gene ,
Wikipedia's
Gene Wiki ,
UniProtKB/Swiss-Prot ,
and/or
UniProtKB/TrEMBL )
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EntrezGene summary for OGDH : This gene encodes one subunit of the 2-oxoglutarate dehydrogenase complex. This complex catalyzesthe overall conversion of 2-oxoglutarate (alpha-ketoglutarate) to succinyl-CoA and CO(2) duringthe Krebs cycle. The protein is located in the mitocondrial matrix and uses thiamine pyrophosphateas a cofactor. A congential deficiency in 2-oxoglutarate dehydrogenase activity is believed tolead to hypotonia, metabolic acidosis, and hyperlactatemia. [provided by RefSeq] UniProtKB/Swiss-Prot: ODO1_HUMAN, Q02218 Function : The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymaticcomponents: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) andlipoamide dehydrogenase (E3)
Genomic Location for OGDH
(According to
GeneLoc and/or
HGNC , and/or
Entrez Gene (NCBI build 36) ,
and/or miRBase ,
Genomic Views according to
UCSC and
Ensembl ,
Transcription factor binding sites according to
SABiosciences )About This Section
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Genomic View : UCSC Golden Path with GeneCards custom track Transcription factor binding sites upstream to the OGDH gene Entrez Gene cytogenetic band: 7p14-p13 Ensembl cytogenetic band: 7p13 HGNC cytogenetic band: 7p13-p11.2 OGDH Gene in genomic location: bands according to Ensembl, locations according to
(and/or Entrez Gene and/or Ensembl if different) GeneLoc gene densities for chromosome 7 GeneLoc Exon Structure
GeneLoc location for GC07P044612:
(about GC identifiers )
Start:
44,612,696 bp from pter
End:
44,715,194 bp from pter
Size:
102,499 bases
Orientation:
plus strand
1 alternative location : Chr 7+,CRA_TCAG 44,685,689-44,788,190
RefSeq DNA sequence: NC_000007.12 NT_007819.16 NT_079592.2 Proteins for OGDH
(According to
1 UniProtKB ,
and/or Ensembl ,
Phosphorylation sites according to 2 Phosphosite ,
RefSeq according to NCBI ,
PDB rendering according to OCA and/or Proteopedia ,
Recombinant Proteins
from Invitrogen ,
Millipore ,
Sigma-Aldrich ,
R&D Systems ,
Enzo Life Sciences ,
Abnova ,
OriGene and/or,
Abcam ,
Biochemical Assays by
Invitrogen ,
Millipore ,
R&D Systems ,
Cell Signaling Technology , and/or
Enzo Life Sciences ,
Ontologies according to Gene
Ontology Consortium 01 Apr 2009 and
Entrez Gene ,
Antibodies by Invitrogen ,
Millipore ,
Sigma-Aldrich ,
R&D Systems ,
Cell Signaling Technology ,
Abcam ,
Abnova , and/or
Novus Biologicals )
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UniProtKB/Swiss-Prot: ODO1_HUMAN, Q02218 (See
protein sequence )Recommended Name: 2-oxoglutarate dehydrogenase E1 component, mitochondrial precursor Size : 1023 amino acids; 115935 Da
Cofactor : Thiamine pyrophosphate
Subcellular location : Mitochondrion matrix
Sequence caution : Sequence=BAA01393.1; Type=Frameshift; Positions=1003; Sequence=BAA06836.1;Type=Frameshift; Positions=1003;
PDB structures from and Proteopedia : 3ERY (3D)
 
Secondary accessions : Q9UDX0
Post-translational modifications:
View phosphorylation sites using PhosphoSite 2
REFSEQ proteins (2 alternative transcripts):
NP_001003941.1 NP_002532.2 ENSEMBL proteins: ENSP00000222673 Human Recombinant Proteins Browse Origene for full length recombinant human proteins expressed in human HEK293 cells 3 Gene Ontology (GO) cellular component terms (links to tree view) :
About this table Antibodies for OGDH: Assays for OGDH:
Protein
Domains/ Families for OGDH(According to InterPro , ProtoNet ,
UniProtKB , and/or BLOCKS ,
Sets of similar genes according to GeneDecks )
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Graphical View of Domain Structure for InterPro Entry Q02218 ProtoNet protein and cluster: Q02218
1 Blocks protein family : IPB005475 Transketolase UniProtKB/Swiss-Prot: ODO1_HUMAN, Q02218 Similarity : Belongs to the alpha-ketoglutarate dehydrogenase family
Gene Function for OGDH
(According to MGI Jun 06 2009, UniProtKB ,
IUBMB ,and/or Genatlas ,
shRNA from
OriGene ,
Sigma-Aldrich , RNAi from
Sigma-Aldrich ,
RNAi Products ,
Clones , and
Q-PCR Products
from Invitrogen ,
Millipore ,
OriGene , and/or
Abnova ,
siRNAs from
Applied Biosystems ,
SYBR primers from OriGene ,
Cell-based Assays from Millipore ,
Ontologies according to Gene Ontology Consortium 01 Apr 2009 via
Entrez Gene .)
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               OriGene 29mer shRNA kit in GFP-retroviral vectors (see all 3 ): NM_002541 Applied Biosystems Silencer ® siRNAs for OGDH Sigma-Aldrich siRNA and siRNA Panels for OGDH Sigma-Aldrich shRNA Panels and shRNA for OGDH Explore Sigma-Aldrich super-pooled esiRNAs                OriGene GFP tagged cDNA clones in CMV expression vector (see all 2 ): NM_001003941                                  Myc/DDK tagged cDNA clones in CMV expression vector (see all 2 ): NM_001003941                                  untagged cDNA clones in CMV expression vector (see all 4 ): BC009580  Primers: Browse
Quantitative PCR Central at Invitrogen for Q-PCR LUX™ Primers               OriGene genome-wide validated SYBR primer pairs: NM_002541 UniProtKB/Swiss-Prot: ODO1_HUMAN, Q02218 Function : The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymaticcomponents: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) andlipoamide dehydrogenase (E3) Catalytic activity : 2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine= [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO(2) Enzyme regulation : Catabolite repressedEnzyme Number (IUBMB): EC 1.2.4.2
Genatlas biochemistry entry for OGDH :2-oxoglutarate lipoamide 2 oxidoreductase (decarboxylating and acceptor-succinylating,component E1of the 2-oxoglutarate complex,mitochondrial matrix 4 Gene Ontology (GO) molecular function terms (links to tree view) :
GO ID Qualified GO term Evidence PubMed IDs GO:0004591 oxoglutarate dehydrogenase (succinyl-transferring) activity
ISS -- GO:0016491 oxidoreductase activity
IEA -- GO:0030976 thiamin pyrophosphate binding
IEA -- GO:0034602 oxoglutarate dehydrogenase (NAD+) activity
IEA --
About this table
Pathways & Interactions for OGDH
(Pathways according to Invitrogen
(maps by GeneGo ),
Millipore ,
Cell Signaling Technology ,
Sigma-Aldrich ,
KEGG
and/or UniProtKB ,
Sets of similar genes according to GeneDecks ,
Proteins Network according to
SABiosciences ,
Interactions according to 1 UniProtKB ,
2 MINT , and/or
3 STRING ,
with links to IntAct and
Ensembl ,
Ontologies according to Gene Ontology Consortium 01 Apr 2009 via
Entrez Gene .)
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4 Sigma-Aldrich "Your Favorite Gene" Pathways for OGDH (Your Favorite Gene powered by Ingenuity) 5/31 Interacting proteins for OGDH (ENSP00000222673 3 ) via UniProtKB, MINT, and/or STRING (see all 31
)About this table 2 Gene Ontology (GO) biological process terms (links to tree view) :
GO ID Qualified GO term Evidence PubMed IDs GO:0006096 glycolysis
IEA -- GO:0055114 oxidation reduction
IEA --
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Drugs & Compounds for OGDH (Chemical Compounds according to UniProtKB , Enzo Life Sciences ,
Sigma-Aldrich , Tocris Bioscience , and/or
Novoseek and Drugs according to
Enzo Life Sciences and/or
PharmGKB )
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Browse Tocris compounds for OGDH 10/32 Novoseek chemical compound relationships for OGDH gene (see all 32
)
Compound
Score
Articles
PubMed IDs for Articles with Shared Sentences (# sentences)
lspfpfdl
86.63
1
11207252 (1)
pyruvate
85.27
80
2188870 (3), 1722146 (2), 8835931 (2), 16338405 (2) (see all 64 )
dihydrolipoamide
84.82
14
1505515 (2), 12464489 (1), 8619544 (1), 16045627 (1) (see all 11 )
2-oxo acid
83.75
8
9089910 (1), 10385636 (1), 7548757 (1), 8617422 (1) (see all 5 )
lipoamide
79.03
10
1722146 (2), 1542694 (2), 12590611 (1), 16045627 (1) (see all 5 )
alpha-ketoadipate
78.16
2
8495733 (2)
nadh
68.74
12
11124972 (2), 2087902 (1), 2117555 (1), 2233821 (1) (see all 9 )
succinic semialdehyde
66.96
1
10812195 (1)
isocitrate
65.30
7
9746330 (1), 17668068 (1), 12668482 (1), 14526533 (1) (see all 6 )
coash
55.20
1
10790315 (1)
About this table
Transcripts for OGDH(GenBank/EMBL/DDBJ Accessions according to
Unigene
(Build 219 Homo sapiens; Jun 2 2009) or GenBank , RefSeq according to Entrez Gene ,
DOTS (version 10), and/or
AceView ,
non coding RNAs according to
RNAdb ,
ESTs according to GeneTide ,
exon structure from GeneLoc ,
alternative splicing isoforms according to ASD and/or
ECgene ,
RNAi Products from Invitrogen ,
Millipore , and/or
Abnova ,
siRNAs from Applied Biosystems ,
Sigma-Aldrich ,
shRNA from
Sigma-Aldrich ,
OriGene ,
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OriGene , Expression Assays from Applied Biosystems )About This Section
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               OriGene 29mer shRNA kit in GFP-retroviral vectors (see all 3 ): NM_002541 Sigma-Aldrich siRNA and siRNA Panels for OGDH Sigma-Aldrich shRNA Panels and shRNA for OGDH Explore Sigma-Aldrich super-pooled esiRNAs
Applied Biosystems Silencer ® siRNAs: NM_001003941 NM_002541
REFSEQ mRNAs for OGDH gene (2 alternative transcripts): NM_001003941.1 NM_002541.2
Applied Biosystems TaqMan ® Gene Expression Assays: NM_001003941 NM_002541
               OriGene GFP tagged cDNA clones in CMV expression vector (see all 2 ): NM_001003941                                  Myc/DDK tagged cDNA clones in CMV expression vector (see all 2 ): NM_001003941                                  untagged cDNA clones in CMV expression vector (see all 4 ): BC009580  
Additional cDNA sequence: AK293871.1 AK294951.1 AK296400.1 AK302805.1 AK304439.1 AK304690.1 AK314179.1 BC004964.1 BC009580.1 BC014617.2 BC128549.1 BC128550.1 CR598912.1 CR610956.1 CR613315.1 CR617563.1 CR617767.1 D10523.1 DQ925673.1 DQ925674.1
24/37 DOTS entries (see all 37
): DT.118120 DT.100791852 DT.97857885 DT.100735380 DT.75200206 DT.121112033 DT.100791854 DT.95155237 DT.100791846 DT.100039132 DT.121112040 DT.40206351 DT.100791848 DT.121112036 DT.40242179 DT.92452428 DT.100791843 DT.121112047 DT.121112054 DT.121112062 DT.97862311 DT.102833542 DT.121112002 DT.121112026
24/500 AceView cDNA sequences (see all 500
):CA396416 BU526945 BM005784 CK724952 AA329688 CA488892 AU133291 BG756625 BQ941210 BQ224918 CR613315 BE744600 BF115199 BM809865 BQ951983 BX331800 BQ954636 BE315070 BQ925256 BU506784 BP337046 CR610956 BE904469 BE384690
highest scoring ESTs for OGDH :AI697389 D10523 AA005109 AA025473 AA095266 AA095571 AA126056 AA159542 AA179435 AA181761
Unigene Cluster for OGDH: Oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) Hs.488181 [show with all ESTs ] Unigene Representative Sequence: NM_002541 GeneLoc Exon Structure 5/17 Alternative Splicing Database (ASD) splice patterns (SP) for OGDH (see all 17
) ExUns: 1a · 1b · 1c · 1d · 1e · 1f ^ 2a · 2b · 2c ^ 3 ^ 4a · 4b · 4c · 4d ^ 5a · 5b · 5c · 5d · 5e ^ 6 ^ 7 ^ 8 ^ 9a · 9b · 9c ^ 10a · SP1 :                                       -     -           SP2 :           -   -   -   -           -             -     -           SP3 :           -   -   -   -   -         -             -     -           SP4 :             -   -   -   -         -             -     -           SP5 :                                                    
ExUns: 10b · 10c · 10d ^ 11a · 11b · 11c · 11d ^ 12 ^ 13a · 13b · 13c ^ 14 ^ 15 ^ 16a · 16b ^ 17 ^ 18a · 18b · 18c ^ 19 ^ 20a · 20b ^ 21 ^ 22 ^ 23 ^ 24 ^ SP1 :             -         -       -                           SP2 :                                                     SP3 :                                                     SP4 :                                                     SP5 :             -                                        
ExUns: 25 ^ 26 ^ 27 SP1 :       SP2 :       SP3 :       SP4 :       SP5 :      
About this scheme ECgene alternative splicing isoforms for OGDH 1 Ensembl transcript including schematic representation : ENST00000222673
Expression for OGDH
(Experimental results according to
1 GeneNote
and GNF BioGPS ,
probe sets-to-genes annotations according to
2 GeneAnnot ,
3 GeneTide ,
Sets of similar genes according to GeneDecks ,
Electronic Northern calculations according to data from
UniGene (Build 219 Homo sapiens),
SAGE tags according to
CGAP ,
plus additional links to
SOURCE , and/or
GNF
BioGPS , and/or
EXPOLDB , and/or
UniProtKB ,
Expression Assays from
Applied Biosystems
)
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OGDH expression in normal and diseased human tissues Applied Biosystems TaqMan ® Gene Expression Assays for OGDH 1 / 2 / 3
6 probe-sets matching OGDH gene Data from
(Publications) and GNF BioGPS About these images About these images CGAP SAGE TAG: GCCAAGGGGCSOURCE GeneReport for Unigene cluster: Hs.488181 Expression variation in blood from EXPOLDB for OGDH
Orthologs for OGDH
(Orthologs according to
1,2 HomoloGene (2 older version, for species not in 1 newer version),
3 euGenes ,
4 SGD
and/or
5 MGI Jun 06 2009,
with possible further links to
Flybase
and/or
WormBase ,
Gene Trees according to Ensembl )
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Orthologs for OGDH gene from 5/24 species (see all 24
)
About this table Species with no ortholog for OGDH ENSEMBL Gene Tree for OGDH Paralogs for OGDH (Paralogs according to 1 HomoloGene and 2 Ensembl , Pseudogenes according to 3 Pseudogene.org )About This Section
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Paralogs for OGDH gene DHTKD1 2 OGDHL 2
SNPs/Variants for OGDH (According to the
1 NCBI SNP Database ,
2 Ensembl ,
3 PupaSUITE , and
UniProtKB ,
Linkage Disequilibrium by HapMap ,
Genotyping Reagents from
Applied Biosystems )
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HapMap Linkage Disequilibrium images for OGDH (up to first 250kb)
Disorders & Mutations for OGDH
(in which this Gene is Involved, According to
OMIM, UniProtKB ,
Novoseek , PharmGKB ,
Genatlas , GeneTests ,
Blood group antigen gene mutations by BGMUT ,
HGMD, GAD ,
HuGE Navigator ,
BCGD ,
and/or TGDB .)
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OMIM: 203740
10/16 Novoseek disease relationships for OGDH gene (see all 16
)
Disease
Score
Articles
PubMed IDs for Articles with Shared Sentences (# sentences)
door syndrome
74.89
1
17343268 (1)
biliary cirrhosis primary
72.67
12
2188870 (2), 9089910 (1), 1380527 (1), 10385636 (1) (see all 11 )
thiamine deficiency
71.36
3
8683340 (1), 19067139 (1)
korsakoff syndrome
54.71
1
8279670 (1)
alzheimers disease
38.44
3
8619544 (1), 2087217 (1), 15663478 (1)
neurodegenerative diseases
38.23
3
19087395 (1), 15840571 (1), 15663478 (1)
neurodegeneration
36.58
2
18783430 (1)
parkinson disease
27.12
3
8986127 (1), 19087395 (1), 12963742 (1)
autoimmune response
15.93
3
8527402 (1)
mimicry
14.82
1
7548757 (1)
About this table Genatlas disease: OGDH congenital lactic acidosis with hypotonia and progressive neurologic deterioration leading toearly death
Medical News for OGDH (Possibly Related Articles in
Doctor's Guide )
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--
Publications for OGDH (in
PubMed .
Associations of this gene to articles via
1 Novoseek ,
2 HGNC ,
3 Entrez Gene ,
4 UniProtKB/Swiss-Prot ,
5 UniProtKB/TrEMBL ,
6 GAD , and/or
7 PharmGKB )
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10/179 PubMed articles for OGDH gene (see all 179
): Cloning and nucleotide sequence of the cDNA encoding human 2-oxoglutarate dehydrogenase (lipoamide). (PubMed id 1542694) 1, 2, 3, 4 Koike K....Goto S. (1992) Localization of the gene (OGDH) coding for the E1k component of the alpha-ketoglutarate dehydrogenase complex to chromosome 7p13-p11.2. (PubMed id 8020988) 1, 2, 3 Szabo P....Blass J.P. (1994) The gene encoding human 2-oxoglutarate dehydrogenase: structural organization and mapping to chromosome 7p13-p14. (PubMed id 7622061) 1, 3, 4 Koike K. (1995) Reduction in the E2k subunit of the alpha-ketoglutarate dehydrogenase complex has effects independent of complex activity. (PubMed id 15649899) 1, 3 Shi Q....Gibson G.E. (2005) Exclusion of OGDH and BMP4 as candidate genes in two siblings with autosomal recessive DOOR syndrome. (PubMed id 17343268) 1, 3 van Bever Y....Courtens W. (2007) Regulation of 2-oxoglutarate (alpha-ketoglutarate) dehydrogenase stability by the RING finger ubiquitin ligase Siah. (PubMed id 15466852) 1, 3 Habelhah H....Ronai Z. (2004) Tryptic digestion of ubiquitin standards reveals an improved strategy for identifying ubiquitinated proteins by mass spectrometry. (PubMed id 17370265) 4 Denis N.J.... Figeys D. (2007) Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes. (PubMed id 16344560) 3 Kimura K.... Sugano S. (2006) Towards a proteome-scale map of the human protein-protein interaction network. (PubMed id 16189514) 3 Rual J.F....Vidal M. (2005) The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). (PubMed id 15489334) 4 Gerhard D.S....Malek J. (2004)
Search for OGDH
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Genome Databases showing OGDH
(According to
Entrez Gene ,
HGNC ,
AceView ,
euGenes ,
Ensembl ,
miRBase ,
ECgene ,
and/or
H-InvDB )
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Other Databases showing OGDH
(According to HUGE )
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Specialized Databases showing OGDH (According to ATLAS , HORDE , IMGT , MTDB, LEIDEN , UniProtKB/Swiss-Prot , and/or UniProtKB/TrEMBL ,Wikipedia and/or GeneReviews via UniProtKB/Swiss-Prot )About This Section
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Name Description
Wikipedia http://en.wikipedia.org/wiki/Alpha-ketoglutarate_dehydrogenase
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-- Services for OGDH (Reagents available from Applied Biosystems , Antibodies and assays by Cell
Signaling Technology , Abcam , Novus Biologicals ,Sigma-Aldrich , R&D Systems , Millipore , Abnova , and/or Invitrogen , Clones available from OriGene ,and/or Invitrogen , Drugs and/or compounds by Sigma-Aldrich , Enzo Life Sciences , and/or Tocris Bioscience )About This Section
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Products for OGDH:
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GeneCards Homepage - Last full update: 2 Jul 2009
Incremental update: 13 Oct 2009