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Category Symbol Source: HGNC EntrezGene Ensembl GeneCards RNA genes CroW21

GIFtS
-

PRODH2 Gene

protein-coding GIFtS: 51
GCID: GC19M036416

proline dehydrogenase (oxidase) 2

Explore 2 diseases affiliated with
PRODH2 via

MalaCards

our new
Human Malady Compendium

(According to ¹HGNC, ²Entrez Gene,
³UniProtKB/Swiss-Prot, ⁴UniProtKB/TrEMBL, ⁵OMIM, ⁶GeneLoc, ⁷Ensembl, ⁸DME, ⁹miRBase, and/or ¹⁰fRNAdb)
About This Section

Aliases
Proline Dehydrogenase (Oxidase) 2¹ ²		Probable Proline Dehydrogenase 2²
HSPOX1¹ ²		HsPOX1³
Kidney And Liver Proline Oxidase 1² ³		PRODH⁵
Probable Proline Oxidase 2² ³		SCZD4⁵
EC 1.5.99.8³ ⁸

External Ids:	HGNC: 17325¹	Entrez Gene: 58510²	Ensembl: ENSG00000250799⁷	OMIM: 606810⁵	UniProtKB: Q9UF12³

Export aliases for PRODH2 gene to outside databases

Previous GC identifers: GC19M036930 GC19M036739 GC19M040967 GC19M040982 GC19M036291 GC19M036292 GC19M032795

(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 PRODH2:

The protein encoded by this gene is similar to proline dehydrogenase (oxidase) 1, a mitochondrial enzyme which

catalyzes the first step in proline catabolism. The function of this protein has not been determined. (provided by

RefSeq, Jul 2008)

UniProtKB/Swiss-Prot: PROD2_HUMAN, Q9UF12

Function: Converts proline to delta-1-pyrroline-5-carboxylate (Probable)

(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 69), Regulatory elements and Epigenetics data according to QIAGEN, SABiosciences, and/or SwitchGear Genomics)
About This Section

RefSeq DNA sequence:

NC_000019.9 NC_018930.1 NT_011109.16

Regulatory elements:

SABiosciences Regulatory transcription factor binding sites in the PRODH2 gene promoter:
USF1 AP-4 RelA Tal-1beta NF-kappaB PPAR-gamma1 USF-1 PPAR-gamma2 NF-kappaB1 ITF-2
Other transcription factors

SwitchGear Promoter luciferase reporter plasmids (see all 2): PRODH2 promoter sequence

Search SABiosciences Chromatin IP Primers for PRODH2

Epigenetics:

QIAGEN PyroMark CpG Assay predesigned Pyrosequencing DNA Methylation assays in human, mouse, rat PRODH2

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

Entrez Gene cytogenetic band: 19q13.1 Ensembl cytogenetic band: 19q13.12 HGNC cytogenetic band: 19q13.1

PRODH2 Gene in genomic location: bands according to Ensembl, locations according to (and/or Entrez Gene and/or Ensembl if different)
PRODH2 gene location

GeneLoc information about chromosome 19 GeneLoc Exon Structure

GeneLoc location for GC19M036416: view genomic region (about GC identifiers)

Start:	36,290,890 bp from pter	End:	36,304,201 bp from pter
Size:	13,312 bases	Orientation:	minus strand

(According to ¹UniProtKB, HORDE, neXtProt, Ensembl, and/or Reactome, Modification sites according to ²PhosphoSitePlus, Specific Peptides from DME, Protein expression images according to data from SPIRE MOPED and PaxDb, 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 Uscn,
Biochemical Assays by EMD Millipore, R&D Systems, OriGene, GenScript, Cell Signaling Technology, Enzo Life Sciences, and/or Uscn, Ontologies according to Gene Ontology Consortium 01 Mar 2013 and Entrez Gene, Antibodies by EMD Millipore, R&D Systems, GenScript, Cell Signaling Technology, OriGene, Novus Biologicals, Thermo Fisher Scientific, Abcam, and/or Uscn)
About This Section

UniProtKB/Swiss-Prot: PROD2_HUMAN, Q9UF12 (See protein sequence)

Recommended Name: Probable proline dehydrogenase 2

Size: 536 amino acids; 58871 Da

Cofactor: FAD (By similarity)

Explore the universe of human proteins at neXtProt for PRODH2: NX_Q9UF12

Post-translational modifications:

View modification sites using PhosphoSitePlus²

View neXtProt modification sites for NX_Q9UF12

4/6 DME Specific Peptides for PRODH2 (Q9UF12) (see all 6)

MVASHNE

KLVRGAY

YGQFVAGET

FGQLLGMCD

PRODH2 Protein expression data from MOPED and PaxDb: --

REFSEQ proteins: NP_067055.1

ENSEMBL proteins:

ENSP00000467089 ENSP00000301175 ENSP00000465214 ENSP00000465310

Human Recombinant Protein Products for PRODH2:

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	Uscn Proteins for PRODH2

Gene Ontology (GO): 2 cellular component terms (GO ID links to tree view): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0005739	mitochondrion	--	--
GO:0005743	mitochondrial inner membrane	IEA	--

PRODH2 for ontologies About GeneDecksing

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(According to InterPro, ProtoNet, UniProtKB, and/or BLOCKS, Sets of similar genes according to GeneDecks)
About This Section

PRODH2 for domains About GeneDecksing

2 InterPro domains/families:

IPR015659 Proline_oxidase

IPR002872 Proline_DH

Graphical View of Domain Structure for InterPro Entry Q9UF12

ProtoNet protein and cluster: Q9UF12

1 Blocks protein family: IPB002872 Proline dehydrogenase

UniProtKB/Swiss-Prot: PROD2_HUMAN, Q9UF12

Similarity: Belongs to the proline oxidase family

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

Molecular Function:

UniProtKB/Swiss-Prot Summary: PROD2_HUMAN, Q9UF12

Function: Converts proline to delta-1-pyrroline-5-carboxylate (Probable)

Catalytic activity: L-proline + acceptor = (S)-1-pyrroline-5-carboxylate + reduced acceptor

Enzyme Number (IUBMB): EC 1.5.99.8¹ ²

Gene Ontology (GO): 1 molecular function term (GO ID links to tree view): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0004657	proline dehydrogenase activity	IEA	--

PRODH2 for ontologies About GeneDecksing

Phenotypes:
2 GenomeRNAi human phenotypes for PRODH2:

Cells with protrusions

Increased gamma-H2AX phosphory

Animal Models:

inGenious Targeting Laboratory - Customizable classic, inducible, and humanized mouse model solutions for PRODH2

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In Situ Assay
Products:

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(Pathways according to EMD Millipore, R&D Systems, Cell Signaling Technology, KEGG, PharmGKB, BioSystems, 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 Mar 2013 via Entrez Gene).
About This Section

Unified GeneCards pathways About this table

See pathways by source

Super-pathway

contained gene-specific pathways

Arginine and proline metabolism

Arginine and proline metabolism

1.00

Metabolism

Metabolic pathways

0.38

Pathway sources
See GeneCards unified pathways
Show all pathways

2 Kegg Pathways (Kegg details for PRODH2):

	Arginine and proline metabolism
	Metabolic pathways

UniProtKB/Swiss-Prot: PROD2_HUMAN, Q9UF12

Pathway: Amino-acid degradation; L-proline degradation into L-glutamate; L-glutamate from L-proline: step 1/2

PRODH2 for pathways About GeneDecksing

Interactions:

Search SABiosciences Gene Network Central^TM Interacting Genes and Proteins Networks for PRODH2

Gene Ontology (GO): 2 biological process terms (GO ID links to tree view): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0006537	glutamate biosynthetic process	IEA	--
GO:0010133	proline catabolic process to glutamate	IEA	--

PRODH2 for ontologies About GeneDecksing

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

Browse Small Molecules at EMD Millipore

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Browse Tocris compounds for PRODH2

1 HMDB Compound for PRODH2 About this table

Compound	Synonyms	CAS #	PubMed Ids
FAD	1H-Purin-6-amine flavin dinucleotide (see all 21)	146-14-5	--

Search CenterWatch for drugs/clinical trials and news about PRODH2 / PROD2

(Secondary structures according to fRNAdb,
GenBank/EMBL/DDBJ Accessions according to
Unigene (Build 235 Homo sapiens; Mar 10 2013) or GenBank,
RefSeq according to Entrez Gene,
DOTS (version 10), and/or AceView, transcript ids from Ensembl with links to UCSC,
exon structure from GeneLoc, alternative splicing isoforms according to ASD and/or ECgene,
RNAi Products from EMD Millipore,
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Tagged/untagged cDNA clones from OriGene, SwitchGear Genomics, GenScript, DNA2.0, Vector BioLabs, Primers from OriGene, SABiosciences, and/or QIAGEN )
About This Section

REFSEQ mRNAs for PRODH2 gene:

NM_021232.1

Unigene Cluster for PRODH2:

Proline dehydrogenase (oxidase) 2

Hs.515366 [show with all ESTs]

Unigene Representative Sequence: AK130040

7 Ensembl transcripts including schematic representations, and UCSC links where relevant:

ENST00000587808 ENST00000592010 ENST00000587695 ENST00000301175(uc002obx.1)

ENST00000588266 ENST00000589835 ENST00000591694

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Additional cDNA sequence:

AK130040.1 BC038853.1 BC039710.1 U80018.1

5 DOTS entries:

DT.207893 DT.95160918 DT.101958357 DT.95160915 DT.95160916

24/33 AceView cDNA sequences (see all 33):

AW771490 BC038853 BG054558 BX955574 U80018 BC039710 NM_021232 AI796255

BX110212 AI927829 BI761466 BI772725 AI640611 AW612108 AI823488 AI700948

BF197299 AI915857 AA203731 AW772333 AI263072 BI766014 BI763429 R02681

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

PRODH2 expression in normal human tissues (normalized intensities)
See probesets specificity/sensitivity at GeneAnnot
About this imageBioGPS
CGAP TAG: --

About this image

PRODH2 expression in embryonic tissues and stem cells
Expression by the Database of Embryonic development, Stem cell research, and Regenerative medicine About this table

4 LifeMap In Vivo Development Anatomical Compartments/Cells

Tissue

Anatomical Compartment

Cell

Category (developmental path)

Kidney

Proximal Tubule

Proximal Tubule Cells

Kidney

Kidney

Kidney

Kidney

Expression:

Positive

Negative

Selective marker

Experimental details:

Curated

Microarrays

In-situ hybridization

Genevestigator expression for PRODH2

SOURCE GeneReport for Unigene cluster: Hs.515366

SABiosciences Expression via Pathway-Focused PCR Array including PRODH2:

Amino Acid Metabolism I in human mouse rat

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In Situ
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(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 eukaryotes.

Orthologs for PRODH2 gene from 6/19 species (see all 19) About this table

Organism	Taxonomic classification	Gene	Description	Human Similarity	Orthology Type	Details
mouse (Mus musculus)	Mammalia	Prodh2^{1 , 5}	proline dehydrogenase (oxidase) 2^{1, 5}	80.83(n)¹ 80.97(a)¹		7 (18.36 cM)⁵ 56189¹ NM_019546.5¹ NP_062419.2¹ 30493622⁵
lizard (Anolis carolinensis)	Reptilia	PRODH2⁶	--	54(a)	1 ↔ 1	GL343287.1(702052-714081)
African clawed frog (Xenopus laevis)	Amphibia	MGC68483²	hypothetical protein MGC68483	74.03(n)		BC059998.1
zebrafish (Danio rerio)	Actinopterygii	57057727²	--	75.69(n)		57057727
thale cress (Arabidopsis thaliana)	eudicotyledons	ERD5¹	proline dehydrogenase 1	41.18(n) 30.88(a)		822833 NM_113981.5 NP_189701.3
rice (Oryza sativa)	Liliopsida	Os10g0550900¹	hypothetical protein	47.91(n) 35.93(a)		4349318 NM_001071853.1 NP_001065321.1

ENSEMBL Gene Tree for PRODH2 (if available)
TreeFam Gene Tree for PRODH2 (if available)

(Paralogs according to ¹HomoloGene,
²Ensembl, and ³SIMAP, Pseudogenes according to ⁴Pseudogene.org Build 68)
About This Section

Paralogs for PRODH2 gene

PRODH²

1 SIMAP similar gene for PRODH2 using alignment to 4 protein entries: PROD2_HUMAN (see all proteins):

PRODH

PRODH2 for paralogs About GeneDecksing

(SNPs/Variants according to the ¹NCBI SNP Database, ²Ensembl, ³PupaSUITE, UniProtKB, 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

SNP ID	Valid	Clinical significance	Chr 19 pos	Sequence	#	AA Chg	Type	More	#	Allele freq	Pop	Total sample	More
Genomic Data					Transcription Related Data				Allele Frequencies
				--	--	--		--	--
rs143685766^1,2		--	36290408(+)	`GGTCTC/TGATCT`	1	--	ds500¹		0	--	--	--	--
rs448434^1,2		--	36290452(+)	`aaactG/Cctggg`	1	--	ds500¹		1		NA	2
rs144546376^1,2		--	36290483(+)	`TGCCCG/TGCCTC`	1	--	ds500¹		0	--	--	--	--
rs200576798^1,2		--	36290500(+)	`TTTTC-/TTTTCT`	1	--	ds500¹		0	--	--	--	--
rs201946606^1,2		--	36290503(+)	`TCTTT-/TCTTTTT`	1	--	ds500¹		0	--	--	--	--
rs139741251^1,2	C	--	36290504(+)	`CTTTT-/CTTTTT`	1	--	ds500¹		0	--	--	--	--
rs73594410^1,2	C	--	36290583(+)	`AGCCTA/CAAACC`	1	--	ds500¹		0	--	--	--	--
rs77233754^1,2	C,F	--	36290705(+)	`CCAGGC/GTGGTC`	1	--	ds500¹		1		EA	120
rs4805139^1,2	C,F,A	--	36290779(+)	`AGAGCT/CACCAC`	1	--	ds500¹		5		NA WA EA	362
rs1133745^1,2	C,F	--	36290900(-)	`CTTAGG/CTGCTG`	1	--	ut3¹		6		NA WA EA	364

HapMap Linkage Disequilibrium report for PRODH2 (36290890 - 36304201 bp)
Structural Variations (Copy Number Variations, Insertions/Deletions, Inversions)
Database of Genomic Variants (DGV) variations for PRODH2: --

SABiosciences Cancer Mutation PCR Assays

Search QIAGEN SeqTarget long-range PCR primers in human, mouse, rat for resequencing PRODH2

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(in which this Gene is Involved, According to MalaCards, OMIM, UniProtKB, the University of Copenhagen DISEASES database, Genatlas, GeneTests, GAD, HuGE Navigator, and/or TGDB.)
About This Section

PRODH2 for disorders           About GeneDecksing

OMIM gene information: 606810
OMIM disorders: 239500  600850
2 diseases for PRODH2:    About MalaCards

hyperprolinemia schizophrenia

Human Genome Epidemiology (HuGE) Navigator: PRODH2 (20 documents)

Export disorders for PRODH2 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 PRODH2 gene integrated from 9 sources:
(articles sorted by number of sources associating them with PRODH2)

Utopia: connect your pdf to the dynamic
world of online information

Genetic variants in nuclear-encoded mitochondrial gen es influence AIDS progression. (PubMed id 20877624)¹ Hendrickson S.L....O'Brien S.J. (2010)
A novel function for hydroxyproline oxidase in apoptosis through generation of reactive oxygen species. (PubMed id 18287100)¹ Cooper S.K....Phang J.M. (2008)
Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. (PubMed id 12477932)¹ Strausberg R.L....Marra M.A. (2002)
Genetic study of schizophrenia: new targets for thera py. (PubMed id 19810846)⁹ Ujike H. and Nakata K. (2003)

(in PubMed, OMIM, and NCBI Bookshelf)
About This Section

		AND	OR
Aliases
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: 58510	HGNC: 17325	AceView: PRODH2	Ensembl:ENSG00000250799	euGenes: HUgn58510
ECgene: PRODH2	Kegg: 58510	H-InvDB: PRODH2

(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

Name	Description
PharmGKB entry for PRODH2	Pharmacogenomics, SNPs, Pathways

Intellectual Property for PRODH2 gene
(Patent information from GeneIP, Licensable technologies from WIS Yeda, Salk, Tufts, IP news from LifeMap Sciences, Inc.)

About This Section

Patent Information for PRODH2 gene:
Search GeneIP for patents involving PRODH2

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, Uscn, Thermo Fisher Scientific, Gene Editing from DNA2.0 and Sirion Biotech, Clones from EMD Millipore, OriGene, GenScript, Sino Biological, DNA2.0, SwitchGear Genomics, Vector BioLabs, Cell lines from GenScript, LifeMap BioReagents, and Sirion Biotech, 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)
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	Browse OriGene Antibodies		OriGene shRNA RFP for PRODH2
	OriGene 29mer shRNA kits in GFP-retroviral vector in human, mouse, rat for PRODH2		OriGene genome-wide validated SYBR primer pairs in human, mouse, rat for PRODH2
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	QIAGEN Custom miScript Target Protector blocks miRNA-binding site of in human, mouse, rat PRODH2
	Search QIAGEN SeqTarget long-range PCR primers in human, mouse, rat for resequencing PRODH2
	QIAGEN PyroMark CpG Assay predesigned Pyrosequencing DNA Methylation assays in human, mouse, rat PRODH2
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	GenScript Custom Purified and Recombinant Proteins Services for PRODH2		GenScript cDNA clones with any tag delivered in your preferred vector for PRODH2
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Vector BioLabs ready-to-use adenovirus/AAV for human, mouse, rat PRODH2

inGenious Targeting Laboratory - Customizable classic, inducible, and humanized mouse model solutions for PRODH2

Sirion Biotech Customized:

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GeneCards Homepage - Last full update: 19 Mar 2013 - Incrementals: 21 Mar 2013 , 15 Apr 2013 , 26 Apr 2013 , 14 May 2013

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Category			VWF (GIFTS: 73) von Willebrand factor
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

The GeneCards Human Gene Database

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

Hot genes Disease genes PRODH2 gene at Home site.

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PRODH2 Gene

proline dehydrogenase (oxidase) 2

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PRODH2 Gene

proline dehydrogenase (oxidase) 2

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for PRODH2 gene

Genomic Views
for PRODH2 gene

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for PRODH2 gene

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Families
for PRODH2 gene

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for PRODH2 gene

Pathways & Interactions
for PRODH2 gene

Drugs & Compounds
for PRODH2 gene

Transcripts
for PRODH2 gene

Expression
for PRODH2 gene

Orthologs
for PRODH2 gene

Paralogs
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Genomic Variants
for PRODH2 gene

Disorders / Diseases
for PRODH2 gene

Publications
for PRODH2 gene

Intellectual Property
for PRODH2 gene

Products
for PRODH2 gene