O43272 (PROD_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Proline dehydrogenase 1, mitochondrial
EC=1.5.99.8

Alternative name(s):
Proline oxidase
Proline oxidase 2
p53-induced gene 6 protein

Gene names

Name:	PRODH
Synonyms:	PIG6, POX2

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	600 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	Converts proline to delta-1-pyrroline-5-carboxylate.
Catalytic activity	L-proline + acceptor = (S)-1-pyrroline-5-carboxylate + reduced acceptor. Ref.3
Cofactor	FAD. Ref.3
Pathway	Amino-acid degradation; L-proline degradation into L-glutamate; L-glutamate from L-proline: step 1/2.
Subcellular location	Mitochondrion matrix.
Tissue specificity	Expressed in lung, skeletal muscle and brain, to a lesser extent in heart and kidney, and weakly in liver, placenta and pancreas.
Induction	During p53/TP53-induced apoptosis. Ref.7
Involvement in disease	Hyperprolinemia 1 (HP-1) [MIM:239500]: Characterized by elevated serum proline levels. May be involved in the psychiatric and behavioral phenotypes associated with the 22q11 velocardiofacial and DiGeorge syndrome. Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.8 Schizophrenia 4 (SCZD4) [MIM:600850]: A complex, multifactorial psychotic disorder or group of disorders characterized by disturbances in the form and content of thought (e.g. delusions, hallucinations), in mood (e.g. inappropriate affect), in sense of self and relationship to the external world (e.g. loss of ego boundaries, withdrawal), and in behavior (e.g bizarre or apparently purposeless behavior). Although it affects emotions, it is distinguished from mood disorders in which such disturbances are primary. Similarly, there may be mild impairment of cognitive function, and it is distinguished from the dementias in which disturbed cognitive function is considered primary. Some patients manifest schizophrenic as well as bipolar disorder symptoms and are often given the diagnosis of schizoaffective disorder. Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. Ref.9
Sequence similarities	Belongs to the proline oxidase family.
Sequence caution	The sequence AAC39529.1 differs from that shown. Reason: Frameshift at positions 540, 563, 568, 577, 580 and 582. The sequence AAH68260.1 differs from that shown. Reason: Frameshift at position 123. The sequence AAH94736.1 differs from that shown. Reason: Frameshift at position 40. The sequence AAH94736.1 differs from that shown. Reason: Artifact. Missing internal sequence that doesn't correspond to an exon-intron boundary. The sequence BAD92709.1 differs from that shown. Reason: Intron retention. Includes intronic sequence at the 5' end.

Ontologies

Keywords
Biological process	Proline metabolism
Cellular component	Mitochondrion
Coding sequence diversity	Alternative splicing Polymorphism
Disease	Disease mutation Schizophrenia
Domain	Transit peptide
Ligand	FAD Flavoprotein
Molecular function	Oxidoreductase
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	4-hydroxyproline catabolic process Traceable author statement PubMed 21998747. Source: BHF-UCL cellular nitrogen compound metabolic process Traceable author statement. Source: Reactome glutamate biosynthetic process Inferred from electronic annotation. Source: InterPro intrinsic apoptotic signaling pathway in response to oxidative stress Non-traceable author statement Ref.7. Source: UniProtKB proline catabolic process Traceable author statement. Source: Reactome proline catabolic process to glutamate Inferred from electronic annotation. Source: UniProtKB-UniPathway
Cellular_component	mitochondrial inner membrane Traceable author statement. Source: Reactome mitochondrial matrix Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular_function	FAD binding Inferred from direct assay Ref.3. Source: UniProtKB proline dehydrogenase activity Inferred from direct assay Ref.3. Source: UniProtKB
Complete GO annotation...

Alternative products

This entry describes 3 isoforms produced by alternative splicing. [Align] [Select]

Isoform 1 (identifier: O43272-4)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

Isoform 2 (identifier: O43272-2)

The sequence of this isoform differs from the canonical sequence as follows:
     1-108: Missing.

Isoform 3 (identifier: O43272-1)

The sequence of this isoform differs from the canonical sequence as follows:
     1-84: Missing.
     85-95: LLARHEQLLYV → MLEFVMREWKK

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – ?		Mitochondrion Potential
	Chain	? – 600		Proline dehydrogenase 1, mitochondrial		PRO_0000025800
Natural variations
	Alternative sequence	1 – 108	108	Missing in isoform 2.		VSP_021848
	Alternative sequence	1 – 84	84	Missing in isoform 3.		VSP_040848
	Alternative sequence	85 – 95	11	LLARHEQLLYV → MLEFVMREWKK in isoform 3.		VSP_040849
	Natural variant	8	1	P → L.		VAR_064883
	Natural variant	19	1	Q → P Moderate reduction of enzymatic activity. Corresponds to variant rs2008720 [ dbSNP | Ensembl ].		VAR_064884
	Natural variant	30	1	P → S.		VAR_064885
	Natural variant	58	1	A → T.		VAR_064886
	Natural variant	167	1	A → V Associated with susceptibility to SCZD4; moderate reduction of enzymatic activity. Ref.3		VAR_029563
	Natural variant	185	1	R → Q No effect on enzymatic activity. Ref.3		VAR_029564
	Natural variant	185	1	R → W Moderate reduction of enzymatic activity. Ref.3 Corresponds to variant rs4819756 [ dbSNP | Ensembl ].		VAR_029565
	Natural variant	275	1	T → N. Ref.11		VAR_029874
	Natural variant	289	1	L → M Can be associated with HP-1 in a subset of schizophrenia patients; no effect on enzymatic activity. Ref.3 Ref.8		VAR_029566
	Natural variant	406	1	P → L Associated with susceptibility to SCZD4; strongly reduced enzymatic activity. Ref.3 Ref.9 Corresponds to variant rs3970555 [ dbSNP | Ensembl ].		VAR_029567
	Natural variant	426	1	D → N Moderate reduction of enzymatic activity. Ref.3		VAR_029568
	Natural variant	427	1	V → M Associated with susceptibility to SCZD4; moderate reduction of enzymatic activity. Ref.3 Ref.9 Corresponds to variant rs2238731 [ dbSNP | Ensembl ].		VAR_029569
	Natural variant	431	1	R → H Associated with HP-1 in a subset of schizophrenia patients; moderate reduction of enzymatic activity. Ref.3 Ref.8 Ref.11 Corresponds to variant rs2904552 [ dbSNP | Ensembl ].		VAR_029570
	Natural variant	441	1	L → P in HP-1; associated with susceptibility to SCZD4; strongly reduced enzymatic activity. Ref.3 Ref.8 Ref.9 Corresponds to variant rs2904551 [ dbSNP | Ensembl ].		VAR_029571
	Natural variant	444	1	G → D. Ref.11		VAR_029875
	Natural variant	453	1	R → C Associated with susceptibility to SCZD4; can be associated with HP-1 in a subset of schizophrenia patients; strongly reduced enzymatic activity. Ref.3 Ref.8 Ref.9 Corresponds to variant rs3970559 [ dbSNP | Ensembl ].		VAR_029572
	Natural variant	455	1	A → S Can be associated with HP-1 in a subset of schizophrenia patients; no effect on enzymatic activity. Ref.3 Ref.8 Corresponds to variant rs1807467 [ dbSNP | Ensembl ].		VAR_029573
	Natural variant	466	1	T → M Associated with susceptibility to SCZD4; strongly reduced affinity for FAD and enzymatic activity. Ref.3 Ref.9 Corresponds to variant rs2870984 [ dbSNP | Ensembl ].		VAR_029574
	Natural variant	472	1	A → T Associated with susceptibility to SCZD4; no effect on enzymatic activity. Ref.3 Ref.8 Ref.9 Corresponds to variant rs2870983 [ dbSNP | Ensembl ].		VAR_029575
	Natural variant	488	1	N → S in a breast cancer sample; somatic mutation. Ref.10		VAR_036566
	Natural variant	521	1	Q → E Strongly reduced enzymatic activity. Ref.3		VAR_029576
	Natural variant	521	1	Q → R Associated with susceptibility to SCZD4; can be associated with HP-1 in a subset of schizophrenia patients; enhanced enzymatic activity. Ref.3 Ref.5 Ref.8 Ref.9 Corresponds to variant rs450046 [ dbSNP | Ensembl ].		VAR_029577
Experimental info
	Sequence conflict	144	1	Y → F in AAH94736. Ref.5
	Sequence conflict	164	1	T → S in AAB88789. Ref.1
	Sequence conflict	343	1	P → H in AAH68260. Ref.5
	Sequence conflict	347	1	R → G in AAF21464. Ref.3
	Sequence conflict	455 – 456	2	AE → Q in AAC39529. Ref.7
	Sequence conflict	571	1	L → F in BAD92709. Ref.6
	Sequence conflict	588	1	R → K in AAC39529. Ref.7
	Sequence conflict	596	1	F → L in AAH68260. Ref.5

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified April 5, 2011. Version 3. Checksum: 249E98A0A5BB8027	FASTA	600	68,002
	10 20 30 40 50 60 MALRRALPAL RPCIPRFVQL STAPASREQP AAGPAAVPGG GSATAVRPPV PAVDFGNAQE 70 80 90 100 110 120 AYRSRRTWEL ARSLLVLRLC AWPALLARHE QLLYVSRKLL GQRLFNKLMK MTFYGHFVAG 130 140 150 160 170 180 EDQESIQPLL RHYRAFGVSA ILDYGVEEDL SPEEAEHKEM ESCTSAAERD GSGTNKRDKQ 190 200 210 220 230 240 YQAHRAFGDR RNGVISARTY FYANEAKCDS HMETFLRCIE ASGRVSDDGF IAIKLTALGR 250 260 270 280 290 300 PQFLLQFSEV LAKWRCFFHQ MAVEQGQAGL AAMDTKLEVA VLQESVAKLG IASRAEIEDW 310 320 330 340 350 360 FTAETLGVSG TMDLLDWSSL IDSRTKLSKH LVVPNAQTGQ LEPLLSRFTE EEELQMTRML 370 380 390 400 410 420 QRMDVLAKKA TEMGVRLMVD AEQTYFQPAI SRLTLEMQRK FNVEKPLIFN TYQCYLKDAY 430 440 450 460 470 480 DNVTLDVELA RREGWCFGAK LVRGAYLAQE RARAAEIGYE DPINPTYEAT NAMYHRCLDY 490 500 510 520 530 540 VLEELKHNAK AKVMVASHNE DTVRFALRRM EELGLHPADH QVYFGQLLGM CDQISFPLGQ 550 560 570 580 590 600 AGYPVYKYVP YGPVMEVLPY LSRRALENSS LMKGTHRERQ LLWLELLRRL RTGNLFHRPA
	Isoform 2 [UniParc]. Checksum: BE51A3277E264431	FASTA	492	56,197
	10 20 30 40 50 60 MKMTFYGHFV AGEDQESIQP LLRHYRAFGV SAILDYGVEE DLSPEEAEHK EMESCTSAAE 70 80 90 100 110 120 RDGSGTNKRD KQYQAHRAFG DRRNGVISAR TYFYANEAKC DSHMETFLRC IEASGRVSDD 130 140 150 160 170 180 GFIAIKLTAL GRPQFLLQFS EVLAKWRCFF HQMAVEQGQA GLAAMDTKLE VAVLQESVAK 190 200 210 220 230 240 LGIASRAEIE DWFTAETLGV SGTMDLLDWS SLIDSRTKLS KHLVVPNAQT GQLEPLLSRF 250 260 270 280 290 300 TEEEELQMTR MLQRMDVLAK KATEMGVRLM VDAEQTYFQP AISRLTLEMQ RKFNVEKPLI 310 320 330 340 350 360 FNTYQCYLKD AYDNVTLDVE LARREGWCFG AKLVRGAYLA QERARAAEIG YEDPINPTYE 370 380 390 400 410 420 ATNAMYHRCL DYVLEELKHN AKAKVMVASH NEDTVRFALR RMEELGLHPA DHQVYFGQLL 430 440 450 460 470 480 GMCDQISFPL GQAGYPVYKY VPYGPVMEVL PYLSRRALEN SSLMKGTHRE RQLLWLELLR 490 RLRTGNLFHR PA
	Isoform 3 [UniParc]. Checksum: E5E72B3C595B4596	FASTA	516	59,231
	10 20 30 40 50 60 MLEFVMREWK KSRKLLGQRL FNKLMKMTFY GHFVAGEDQE SIQPLLRHYR AFGVSAILDY 70 80 90 100 110 120 GVEEDLSPEE AEHKEMESCT SAAERDGSGT NKRDKQYQAH RAFGDRRNGV ISARTYFYAN 130 140 150 160 170 180 EAKCDSHMET FLRCIEASGR VSDDGFIAIK LTALGRPQFL LQFSEVLAKW RCFFHQMAVE 190 200 210 220 230 240 QGQAGLAAMD TKLEVAVLQE SVAKLGIASR AEIEDWFTAE TLGVSGTMDL LDWSSLIDSR 250 260 270 280 290 300 TKLSKHLVVP NAQTGQLEPL LSRFTEEEEL QMTRMLQRMD VLAKKATEMG VRLMVDAEQT 310 320 330 340 350 360 YFQPAISRLT LEMQRKFNVE KPLIFNTYQC YLKDAYDNVT LDVELARREG WCFGAKLVRG 370 380 390 400 410 420 AYLAQERARA AEIGYEDPIN PTYEATNAMY HRCLDYVLEE LKHNAKAKVM VASHNEDTVR 430 440 450 460 470 480 FALRRMEELG LHPADHQVYF GQLLGMCDQI SFPLGQAGYP VYKYVPYGPV MEVLPYLSRR 490 500 510 ALENSSLMKG THRERQLLWL ELLRRLRTGN LFHRPA

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"A human homologue of the Drosophila melanogaster sluggish-A (proline oxidase) gene maps to 22q11.2, and is a candidate gene for type-I hyperprolinaemia." Campbell H.D., Webb G.C., Young I.G. Hum. Genet. 101:69-74(1997) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 3). Tissue: Brain.
[2]	"The gene encoding proline dehydrogenase modulates sensorimotor gating in mice." Gogos J.A., Santha M., Takacs Z., Beck K.D., Luine V., Lucas L.R., Nadler J.V., Karayiorgou M. Nat. Genet. 21:434-439(1999) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 3). Tissue: Cerebellum and Kidney.
[3]	"Functional consequences of PRODH missense mutations." Bender H.-U., Almashanu S., Steel G., Hu C.-A., Lin W.-W., Willis A., Pulver A., Valle D. Am. J. Hum. Genet. 76:409-420(2005) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), ENZYME ACTIVITY, COFACTOR, VARIANTS VAL-167 AND ARG-521, CHARACTERIZATION OF VARIANTS VAL-167; TRP-185; GLN-185; MET-289; LEU-406; ASN-426; MET-427; HIS-431; PRO-441; CYS-453; SER-455; MET-466; THR-472; ARG-521 AND GLU-521. Tissue: Kidney.
[4]	"The DNA sequence of human chromosome 22." Dunham I., Hunt A.R., Collins J.E., Bruskiewich R., Beare D.M., Clamp M., Smink L.J., Ainscough R., Almeida J.P., Babbage A.K., Bagguley C., Bailey J., Barlow K.F., Bates K.N., Beasley O.P., Bird C.P., Blakey S.E., Bridgeman A.M. , Buck D., Burgess J., Burrill W.D., Burton J., Carder C., Carter N.P., Chen Y., Clark G., Clegg S.M., Cobley V.E., Cole C.G., Collier R.E., Connor R., Conroy D., Corby N.R., Coville G.J., Cox A.V., Davis J., Dawson E., Dhami P.D., Dockree C., Dodsworth S.J., Durbin R.M., Ellington A.G., Evans K.L., Fey J.M., Fleming K., French L., Garner A.A., Gilbert J.G.R., Goward M.E., Grafham D.V., Griffiths M.N.D., Hall C., Hall R.E., Hall-Tamlyn G., Heathcott R.W., Ho S., Holmes S., Hunt S.E., Jones M.C., Kershaw J., Kimberley A.M., King A., Laird G.K., Langford C.F., Leversha M.A., Lloyd C., Lloyd D.M., Martyn I.D., Mashreghi-Mohammadi M., Matthews L.H., Mccann O.T., Mcclay J., Mclaren S., McMurray A.A., Milne S.A., Mortimore B.J., Odell C.N., Pavitt R., Pearce A.V., Pearson D., Phillimore B.J.C.T., Phillips S.H., Plumb R.W., Ramsay H., Ramsey Y., Rogers L., Ross M.T., Scott C.E., Sehra H.K., Skuce C.D., Smalley S., Smith M.L., Soderlund C., Spragon L., Steward C.A., Sulston J.E., Swann R.M., Vaudin M., Wall M., Wallis J.M., Whiteley M.N., Willey D.L., Williams L., Williams S.A., Williamson H., Wilmer T.E., Wilming L., Wright C.L., Hubbard T., Bentley D.R., Beck S., Rogers J., Shimizu N., Minoshima S., Kawasaki K., Sasaki T., Asakawa S., Kudoh J., Shintani A., Shibuya K., Yoshizaki Y., Aoki N., Mitsuyama S., Roe B.A., Chen F., Chu L., Crabtree J., Deschamps S., Do A., Do T., Dorman A., Fang F., Fu Y., Hu P., Hua A., Kenton S., Lai H., Lao H.I., Lewis J., Lewis S., Lin S.-P., Loh P., Malaj E., Nguyen T., Pan H., Phan S., Qi S., Qian Y., Ray L., Ren Q., Shaull S., Sloan D., Song L., Wang Q., Wang Y., Wang Z., White J., Willingham D., Wu H., Yao Z., Zhan M., Zhang G., Chissoe S., Murray J., Miller N., Minx P., Fulton R., Johnson D., Bemis G., Bentley D., Bradshaw H., Bourne S., Cordes M., Du Z., Fulton L., Goela D., Graves T., Hawkins J., Hinds K., Kemp K., Latreille P., Layman D., Ozersky P., Rohlfing T., Scheet P., Walker C., Wamsley A., Wohldmann P., Pepin K., Nelson J., Korf I., Bedell J.A., Hillier L.W., Mardis E., Waterston R., Wilson R., Emanuel B.S., Shaikh T., Kurahashi H., Saitta S., Budarf M.L., McDermid H.E., Johnson A., Wong A.C.C., Morrow B.E., Edelmann L., Kim U.J., Shizuya H., Simon M.I., Dumanski J.P., Peyrard M., Kedra D., Seroussi E., Fransson I., Tapia I., Bruder C.E., O'Brien K.P., Wilkinson P., Bodenteich A., Hartman K., Hu X., Khan A.S., Lane L., Tilahun Y., Wright H. Nature 402:489-495(1999) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2), VARIANT ARG-521. Tissue: Brain.
[6]	Totoki Y., Toyoda A., Takeda T., Sakaki Y., Tanaka A., Yokoyama S., Ohara O., Nagase T., Kikuno R.F. Submitted (MAR-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 337-600 (ISOFORMS 1/2/3). Tissue: Brain.
[7]	"A model for p53-induced apoptosis." Polyak K., Xia Y., Zweier J.L., Kinzler K.W., Vogelstein B. Nature 389:300-306(1997) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 444-600, INDUCTION BY TP53.
[8]	"PRODH mutations and hyperprolinemia in a subset of schizophrenic patients." Jacquet H., Raux G., Thibaut F., Hecketsweiler B., Houy E., Demilly C., Haouzir S., Allio G., Fouldrin G., Drouin V., Bou J., Petit M., Campion D., Frebourg T. Hum. Mol. Genet. 11:2243-2249(2002) [PubMed] [Europe PMC] [Abstract] Cited for: VARIANTS MET-289; HIS-431; PRO-441; CYS-453; SER-455; THR-472 AND ARG-521, INVOLVEMENT IN HP-1.
[9]	"Genetic variation at the 22q11 PRODH2/DGCR6 locus presents an unusual pattern and increases susceptibility to schizophrenia." Liu H., Heath S.C., Sobin C., Roos J.L., Galke B.L., Blundell M.L., Lenane M., Robertson B., Wijsman E.M., Rapoport J.L., Gogos J.A., Karayiorgou M. Proc. Natl. Acad. Sci. U.S.A. 99:3717-3722(2002) [PubMed] [Europe PMC] [Abstract] Cited for: VARIANTS LEU-406; MET-427; PRO-441; CYS-453; MET-466; THR-472 AND ARG-521, INVOLVEMENT IN SCZD4.
[10]	"The consensus coding sequences of human breast and colorectal cancers." Sjoeblom T., Jones S., Wood L.D., Parsons D.W., Lin J., Barber T.D., Mandelker D., Leary R.J., Ptak J., Silliman N., Szabo S., Buckhaults P., Farrell C., Meeh P., Markowitz S.D., Willis J., Dawson D., Willson J.K.V. , Gazdar A.F., Hartigan J., Wu L., Liu C., Parmigiani G., Park B.H., Bachman K.E., Papadopoulos N., Vogelstein B., Kinzler K.W., Velculescu V.E. Science 314:268-274(2006) [PubMed] [Europe PMC] [Abstract] Cited for: VARIANT [LARGE SCALE ANALYSIS] SER-488.
[11]	"Involvement of hyperprolinemia in cognitive and psychiatric features of the 22q11 deletion syndrome." Raux G., Bumsel E., Hecketsweiler B., van Amelsvoort T., Zinkstok J., Manouvrier-Hanu S., Fantini C., Breviere G.-M.M., Di Rosa G., Pustorino G., Vogels A., Swillen A., Legallic S., Bou J., Opolczynski G., Drouin-Garraud V., Lemarchand M., Philip N. , Gerard-Desplanches A., Carlier M., Philippe A., Nolen M.C., Heron D., Sarda P., Lacombe D., Coizet C., Alembik Y., Layet V., Afenjar A., Hannequin D., Demily C., Petit M., Thibaut F., Frebourg T., Campion D. Hum. Mol. Genet. 16:83-91(2007) [PubMed] [Europe PMC] [Abstract] Cited for: VARIANTS ASN-275; HIS-431 AND ASP-444.
+	Additional computationally mapped references.

Web resources

GeneReviews

Cross-references

Sequence databases
EMBL GenBank DDBJ	U82381 mRNA. Translation: AAB88789.1. AF120278 mRNA. Translation: AAD24775.1. U79754 mRNA. Translation: AAF21464.1. AC007326 Genomic DNA. No translation available. BC068260 mRNA. Translation: AAH68260.1. Frameshift. BC094736 mRNA. Translation: AAH94736.1. Sequence problems. BC118597 mRNA. Translation: AAI18598.1. BC121809 mRNA. Translation: AAI21810.1. AB209472 mRNA. Translation: BAD92709.1. Sequence problems. AF010310 mRNA. Translation: AAC39529.1. Frameshift.
IPI	IPI00383798. IPI00816398. IPI00816668.
RefSeq	NP_001182155.1. NM_001195226.1. NP_057419.4. NM_016335.4.
UniGene	Hs.517352.
3D structure databases
ProteinModelPortal	O43272.
ModBase	Search...
PTM databases
PhosphoSite	O43272.
Proteomic databases
PaxDb	O43272.
PRIDE	O43272.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000357068; ENSP00000349577; ENSG00000100033.
GeneID	5625.
KEGG	hsa:5625.
UCSC	uc002zok.4. human.
Organism-specific databases
CTD	5625.
GeneCards	GC22M018900.
HGNC	HGNC:9453. PRODH.
HPA	HPA020361.
MIM	181500. phenotype. 239500. phenotype. 600850. phenotype. 606810. gene.
neXtProt	NX_O43272.
Orphanet	419. Hyperprolinemia type 1. 3140. Schizophrenia.
PharmGKB	PA33801.
GenAtlas	Search...
Phylogenomic databases
eggNOG	COG0506.
HOVERGEN	HBG008262.
InParanoid	O43272.
KO	K00318.
OrthoDB	EOG41G35C.
Enzyme and pathway databases
BioCyc	MetaCyc:HS01958-MONOMER.
Reactome	REACT_111217. Metabolism.
UniPathway	UPA00261; UER00373.
Gene expression databases
ArrayExpress	O43272.
Bgee	O43272.
Genevestigator	O43272.
GermOnline	ENSG00000100033. Homo sapiens.
Family and domain databases
InterPro	IPR002872. Proline_DH. IPR015659. Proline_oxidase. [Graphical view]
PANTHER	PTHR13914. PTHR13914. 1 hit.
Pfam	PF01619. Pro_dh. 1 hit. [Graphical view]
ProtoNet	Search...
Other
ChiTaRS	PRODH. human.
DrugBank	DB00172. L-Proline.
GenomeRNAi	5625.
NextBio	21864.
SOURCE	Search...

Entry information

Entry name

PROD_HUMAN

Accession

Primary (citable) accession number: O43272
Secondary accession number(s): A6NF53 , O14680, Q0P507, Q147W8, Q504W1, Q59FI8, Q6NV86, Q9UF13

Entry history

Integrated into UniProtKB/Swiss-Prot:	May 30, 2000
Last sequence update:	April 5, 2011
Last modified:	May 1, 2013
This is version 123 of the entry and version 3 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.