Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Proline dehydrogenase 1, mitochondrial

Gene

PRODH

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Converts proline to delta-1-pyrroline-5-carboxylate.

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the ‘Function’ section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

FAD1 Publication

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: L-proline degradation into L-glutamate

This protein is involved in step 1 of the subpathway that synthesizes L-glutamate from L-proline.
Proteins known to be involved in the 2 steps of the subpathway in this organism are:
  1. Proline dehydrogenase 1, mitochondrial (PRODH)
  2. Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial (ALDH4A1)
This subpathway is part of the pathway L-proline degradation into L-glutamate, which is itself part of Amino-acid degradation.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes L-glutamate from L-proline, the pathway L-proline degradation into L-glutamate and in Amino-acid degradation.

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

  • FAD binding Source: UniProtKB
  • proline dehydrogenase activity Source: UniProtKB

GO - Biological processi

  • 4-hydroxyproline catabolic process Source: BHF-UCL
  • intrinsic apoptotic signaling pathway in response to oxidative stress Source: UniProtKB
  • positive regulation of cell death Source: ParkinsonsUK-UCL
  • proline catabolic process Source: Reactome
  • proline catabolic process to glutamate Source: GO_Central
  • proline metabolic process Source: ProtInc

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionOxidoreductase
Biological processProline metabolism
LigandFAD, Flavoprotein

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

More...
BioCyci
MetaCyc:HS01958-MONOMER

BRENDA Comprehensive Enzyme Information System

More...
BRENDAi
1.5.99.B2 2681

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-70688 Proline catabolism

SIGNOR Signaling Network Open Resource

More...
SIGNORi
O43272

UniPathway: a resource for the exploration and annotation of metabolic pathways

More...
UniPathwayi
UPA00261;UER00373

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Proline dehydrogenase 1, mitochondrialCurated (EC:1.5.5.21 Publication)
Alternative name(s):
Proline oxidase
Proline oxidase 2
p53-induced gene 6 protein
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:PRODHImported
Synonyms:PIG6, POX2, PRODH21 Publication
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 22

Organism-specific databases

Eukaryotic Pathogen Database Resources

More...
EuPathDBi
HostDB:ENSG00000100033.16

Human Gene Nomenclature Database

More...
HGNCi
HGNC:9453 PRODH

Online Mendelian Inheritance in Man (OMIM)

More...
MIMi
606810 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_O43272

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Hyperprolinemia 1 (HYRPRO1)3 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn inborn error of proline metabolism resulting in elevated levels of proline in the plasma and urine. The disorder is generally benign and most affected individuals are clinically asymptomatic. Some patients, however, have neurologic manifestations, including epilepsy and mental retardation. Association with certain forms of schizophrenia have been reported.
See also OMIM:239500
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_029566289L → M in HYRPRO1; mild decrease of enzymatic activity. 2 Publications1
Natural variantiVAR_029568426D → N in HYRPRO1; moderate reduction of enzymatic activity. 1 Publication1
Natural variantiVAR_029569427V → M in HYRPRO1 and SCZD4; may be associated with disease susceptibility; moderate reduction of enzymatic activity. 2 PublicationsCorresponds to variant dbSNP:rs2238731EnsemblClinVar.1
Natural variantiVAR_029570431R → H in HYRPRO1; moderate reduction of enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs2904552EnsemblClinVar.1
Natural variantiVAR_029571441L → P in HYRPRO1 and SCZD4; may be associated with disease susceptibility; strongly reduced enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs2904551EnsemblClinVar.1
Natural variantiVAR_029572453R → C in HYRPRO1 and SCZD4; may be associated with disease susceptibility; strongly reduced enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs3970559EnsemblClinVar.1
Natural variantiVAR_029573455A → S in HYRPRO1; mild decrease of enzymatic activity. 2 PublicationsCorresponds to variant dbSNP:rs1807467EnsemblClinVar.1
Natural variantiVAR_029575472A → T in HYRPRO1 and SCZD4; associated with disease susceptibility; mild decrease of enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs2870983Ensembl.1
Natural variantiVAR_029577521Q → R in HYRPRO1 and SCZD4; may be associated with disease susceptibility; enhanced enzymatic activity. 4 PublicationsCorresponds to variant dbSNP:rs450046EnsemblClinVar.1
Schizophrenia 4 (SCZD4)2 Publications
Disease susceptibility is associated with variations affecting the gene represented in this entry.
Disease descriptionA 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.
See also OMIM:600850
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_029567406P → L in SCZD4; may be associated with disease susceptibility; strongly reduced enzymatic activity. 2 PublicationsCorresponds to variant dbSNP:rs3970555Ensembl.1
Natural variantiVAR_029569427V → M in HYRPRO1 and SCZD4; may be associated with disease susceptibility; moderate reduction of enzymatic activity. 2 PublicationsCorresponds to variant dbSNP:rs2238731EnsemblClinVar.1
Natural variantiVAR_029571441L → P in HYRPRO1 and SCZD4; may be associated with disease susceptibility; strongly reduced enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs2904551EnsemblClinVar.1
Natural variantiVAR_029572453R → C in HYRPRO1 and SCZD4; may be associated with disease susceptibility; strongly reduced enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs3970559EnsemblClinVar.1
Natural variantiVAR_029574466T → M in SCZD4; may be associated with disease susceptibility; strongly reduced affinity for FAD; strongly reduced enzymatic activity. 2 PublicationsCorresponds to variant dbSNP:rs2870984EnsemblClinVar.1
Natural variantiVAR_029575472A → T in HYRPRO1 and SCZD4; associated with disease susceptibility; mild decrease of enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs2870983Ensembl.1
Natural variantiVAR_029577521Q → R in HYRPRO1 and SCZD4; may be associated with disease susceptibility; enhanced enzymatic activity. 4 PublicationsCorresponds to variant dbSNP:rs450046EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Schizophrenia

Organism-specific databases

DisGeNET

More...
DisGeNETi
5625

MalaCards human disease database

More...
MalaCardsi
PRODH
MIMi181500 phenotype
239500 phenotype
600850 phenotype

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
419 Hyperprolinemia type 1

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...
PharmGKBi
PA33801

Chemistry databases

Drug and drug target database

More...
DrugBanki
DB06756 Glycine betaine
DB00172 L-Proline

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
PRODH

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_0000025800? – 600Proline dehydrogenase 1, mitochondrial
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – ?MitochondrionSequence analysis

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei368N6-acetyllysineBy similarity1
Modified residuei486N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

Encyclopedia of Proteome Dynamics

More...
EPDi
O43272

jPOST - Japan Proteome Standard Repository/Database

More...
jPOSTi
O43272

PaxDb, a database of protein abundance averages across all three domains of life

More...
PaxDbi
O43272

PeptideAtlas

More...
PeptideAtlasi
O43272

PRoteomics IDEntifications database

More...
PRIDEi
O43272

ProteomicsDB human proteome resource

More...
ProteomicsDBi
48843
48844 [O43272-1]
48845 [O43272-2]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
O43272

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...
PhosphoSitePlusi
O43272

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Expressed in lung, skeletal muscle and brain, to a lesser extent in heart and kidney, and weakly in liver, placenta and pancreas.

<p>This subsection of the ‘Expression’ section reports the experimentally proven effects of inducers and repressors (usually chemical compounds or environmental factors) on the level of protein (or mRNA) expression (up-regulation, down-regulation, constitutive expression).<p><a href='/help/induction' target='_top'>More...</a></p>Inductioni

During p53/TP53-induced apoptosis.1 Publication

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...
Bgeei
ENSG00000100033 Expressed in 89 organ(s), highest expression level in right lung

ExpressionAtlas, Differential and Baseline Expression

More...
ExpressionAtlasi
O43272 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...
Genevisiblei
O43272 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
HPA020361

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

More...
BioGridi
111609, 9 interactors

STRING: functional protein association networks

More...
STRINGi
9606.ENSP00000349577

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

More...
ProteinModelPortali
O43272

Database of comparative protein structure models

More...
ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

More...
MobiDBi
Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the proline oxidase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...
eggNOGi
KOG0186 Eukaryota
COG0506 LUCA

The HOVERGEN Database of Homologous Vertebrate Genes

More...
HOVERGENi
HBG008262

InParanoid: Eukaryotic Ortholog Groups

More...
InParanoidi
O43272

KEGG Orthology (KO)

More...
KOi
K00318

Database of Orthologous Groups

More...
OrthoDBi
912212at2759

Database for complete collections of gene phylogenies

More...
PhylomeDBi
O43272

TreeFam database of animal gene trees

More...
TreeFami
TF313544

Family and domain databases

Integrated resource of protein families, domains and functional sites

More...
InterProi
View protein in InterPro
IPR029041 FAD-linked_oxidoreductase-like
IPR002872 Proline_DH_dom
IPR015659 Proline_oxidase

The PANTHER Classification System

More...
PANTHERi
PTHR13914 PTHR13914, 1 hit

Pfam protein domain database

More...
Pfami
View protein in Pfam
PF01619 Pro_dh, 1 hit

Superfamily database of structural and functional annotation

More...
SUPFAMi
SSF51730 SSF51730, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (3+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 3 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 3 described isoforms and 5 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: O43272-4) [UniParc]FASTAAdd to basket

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.

« Hide
        10         20         30         40         50
MALRRALPAL RPCIPRFVQL STAPASREQP AAGPAAVPGG GSATAVRPPV
60 70 80 90 100
PAVDFGNAQE AYRSRRTWEL ARSLLVLRLC AWPALLARHE QLLYVSRKLL
110 120 130 140 150
GQRLFNKLMK MTFYGHFVAG EDQESIQPLL RHYRAFGVSA ILDYGVEEDL
160 170 180 190 200
SPEEAEHKEM ESCTSAAERD GSGTNKRDKQ YQAHRAFGDR RNGVISARTY
210 220 230 240 250
FYANEAKCDS HMETFLRCIE ASGRVSDDGF IAIKLTALGR PQFLLQFSEV
260 270 280 290 300
LAKWRCFFHQ MAVEQGQAGL AAMDTKLEVA VLQESVAKLG IASRAEIEDW
310 320 330 340 350
FTAETLGVSG TMDLLDWSSL IDSRTKLSKH LVVPNAQTGQ LEPLLSRFTE
360 370 380 390 400
EEELQMTRML QRMDVLAKKA TEMGVRLMVD AEQTYFQPAI SRLTLEMQRK
410 420 430 440 450
FNVEKPLIFN TYQCYLKDAY DNVTLDVELA RREGWCFGAK LVRGAYLAQE
460 470 480 490 500
RARAAEIGYE DPINPTYEAT NAMYHRCLDY VLEELKHNAK AKVMVASHNE
510 520 530 540 550
DTVRFALRRM EELGLHPADH QVYFGQLLGM CDQISFPLGQ AGYPVYKYVP
560 570 580 590 600
YGPVMEVLPY LSRRALENSS LMKGTHRERQ LLWLELLRRL RTGNLFHRPA
Length:600
Mass (Da):68,002
Last modified:April 5, 2011 - v3
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i249E98A0A5BB8027
GO
Isoform 2 (identifier: O43272-2) [UniParc]FASTAAdd to basket

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

Show »
Length:492
Mass (Da):56,197
Checksum:iBE51A3277E264431
GO
Isoform 3 (identifier: O43272-1) [UniParc]FASTAAdd to basket

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

Show »
Length:516
Mass (Da):59,231
Checksum:iE5E72B3C595B4596
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 5 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A087WWM6A0A087WWM6_HUMAN
Proline dehydrogenase
PRODH
600Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9JIW4C9JIW4_HUMAN
Proline dehydrogenase
PRODH
135Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E7EQL6E7EQL6_HUMAN
Proline dehydrogenase
PRODH
492Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H7C363H7C363_HUMAN
Proline dehydrogenase 1, mitochondr...
PRODH
185Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H7C3U9H7C3U9_HUMAN
Proline dehydrogenase 1, mitochondr...
PRODH
164Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

The sequence AAC39529 differs from that shown. Reason: Frameshift at positions 540, 563, 568, 577, 580 and 582.Curated
The sequence AAH68260 differs from that shown. Reason: Frameshift at position 123.Curated
The sequence AAH94736 differs from that shown. Artifact. Missing internal sequence that doesn't correspond to an exon-intron boundary.Curated
The sequence AAH94736 differs from that shown. Reason: Frameshift at position 40.Curated
The sequence BAD92709 differs from that shown. Intron retention. Includes intronic sequence at the 5' end.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti144Y → F in AAH94736 (PubMed:15489334).Curated1
Sequence conflicti164T → S in AAB88789 (PubMed:9385373).Curated1
Sequence conflicti343P → H in AAH68260 (PubMed:15489334).Curated1
Sequence conflicti347R → G in AAF21464 (PubMed:15662599).Curated1
Sequence conflicti455 – 456AE → Q in AAC39529 (PubMed:9305847).Curated2
Sequence conflicti571L → F in BAD92709 (Ref. 6) Curated1
Sequence conflicti588R → K in AAC39529 (PubMed:9305847).Curated1
Sequence conflicti596F → L in AAH68260 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0648838P → L. 1
Natural variantiVAR_06488419Q → P Polymorphism; moderate reduction of enzymatic activity. Corresponds to variant dbSNP:rs2008720Ensembl.1
Natural variantiVAR_06488530P → S. 1
Natural variantiVAR_06488658A → T. 1
Natural variantiVAR_029563167A → V Polymorphism; may be a risk factor for schizophrenia; moderate reduction of enzymatic activity. 1 Publication1
Natural variantiVAR_029564185R → Q Polymorphism; mild decrease of enzymatic activity. 1 Publication1
Natural variantiVAR_029565185R → W Polymorphism; moderate reduction of enzymatic activity. 1 PublicationCorresponds to variant dbSNP:rs4819756EnsemblClinVar.1
Natural variantiVAR_029874275T → N1 Publication1
Natural variantiVAR_029566289L → M in HYRPRO1; mild decrease of enzymatic activity. 2 Publications1
Natural variantiVAR_029567406P → L in SCZD4; may be associated with disease susceptibility; strongly reduced enzymatic activity. 2 PublicationsCorresponds to variant dbSNP:rs3970555Ensembl.1
Natural variantiVAR_029568426D → N in HYRPRO1; moderate reduction of enzymatic activity. 1 Publication1
Natural variantiVAR_029569427V → M in HYRPRO1 and SCZD4; may be associated with disease susceptibility; moderate reduction of enzymatic activity. 2 PublicationsCorresponds to variant dbSNP:rs2238731EnsemblClinVar.1
Natural variantiVAR_029570431R → H in HYRPRO1; moderate reduction of enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs2904552EnsemblClinVar.1
Natural variantiVAR_029571441L → P in HYRPRO1 and SCZD4; may be associated with disease susceptibility; strongly reduced enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs2904551EnsemblClinVar.1
Natural variantiVAR_029875444G → D1 Publication1
Natural variantiVAR_029572453R → C in HYRPRO1 and SCZD4; may be associated with disease susceptibility; strongly reduced enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs3970559EnsemblClinVar.1
Natural variantiVAR_029573455A → S in HYRPRO1; mild decrease of enzymatic activity. 2 PublicationsCorresponds to variant dbSNP:rs1807467EnsemblClinVar.1
Natural variantiVAR_029574466T → M in SCZD4; may be associated with disease susceptibility; strongly reduced affinity for FAD; strongly reduced enzymatic activity. 2 PublicationsCorresponds to variant dbSNP:rs2870984EnsemblClinVar.1
Natural variantiVAR_029575472A → T in HYRPRO1 and SCZD4; associated with disease susceptibility; mild decrease of enzymatic activity. 3 PublicationsCorresponds to variant dbSNP:rs2870983Ensembl.1
Natural variantiVAR_036566488N → S in a breast cancer sample; somatic mutation. 1 Publication1
Natural variantiVAR_029576521Q → E Strongly reduced enzymatic activity. 1 Publication1
Natural variantiVAR_029577521Q → R in HYRPRO1 and SCZD4; may be associated with disease susceptibility; enhanced enzymatic activity. 4 PublicationsCorresponds to variant dbSNP:rs450046EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting. The information stored in this subsection is used to automatically construct alternative protein sequence(s) for display.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_0218481 – 108Missing in isoform 2. 1 PublicationAdd BLAST108
Alternative sequenceiVSP_0408481 – 84Missing in isoform 3. 2 PublicationsAdd BLAST84
Alternative sequenceiVSP_04084985 – 95LLARHEQLLYV → MLEFVMREWKK in isoform 3. 2 PublicationsAdd BLAST11

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...
EMBLi

GenBank nucleotide sequence database

More...
GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...
DDBJi
Links Updated
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.

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS13754.1 [O43272-4]
CCDS56223.1 [O43272-2]

NCBI Reference Sequences

More...
RefSeqi
NP_001182155.1, NM_001195226.1 [O43272-2]
NP_057419.4, NM_016335.4

UniGene gene-oriented nucleotide sequence clusters

More...
UniGenei
Hs.517352

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000357068; ENSP00000349577; ENSG00000100033

Database of genes from NCBI RefSeq genomes

More...
GeneIDi
5625

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:5625

UCSC genome browser

More...
UCSCi
uc062bjw.1 human [O43272-4]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
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.
CCDSiCCDS13754.1 [O43272-4]
CCDS56223.1 [O43272-2]
RefSeqiNP_001182155.1, NM_001195226.1 [O43272-2]
NP_057419.4, NM_016335.4
UniGeneiHs.517352

3D structure databases

ProteinModelPortaliO43272
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111609, 9 interactors
STRINGi9606.ENSP00000349577

Chemistry databases

DrugBankiDB06756 Glycine betaine
DB00172 L-Proline

PTM databases

iPTMnetiO43272
PhosphoSitePlusiO43272

Polymorphism and mutation databases

BioMutaiPRODH

Proteomic databases

EPDiO43272
jPOSTiO43272
PaxDbiO43272
PeptideAtlasiO43272
PRIDEiO43272
ProteomicsDBi48843
48844 [O43272-1]
48845 [O43272-2]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000357068; ENSP00000349577; ENSG00000100033
GeneIDi5625
KEGGihsa:5625
UCSCiuc062bjw.1 human [O43272-4]

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
5625
DisGeNETi5625
EuPathDBiHostDB:ENSG00000100033.16

GeneCards: human genes, protein and diseases

More...
GeneCardsi
PRODH
HGNCiHGNC:9453 PRODH
HPAiHPA020361
MalaCardsiPRODH
MIMi181500 phenotype
239500 phenotype
600850 phenotype
606810 gene
neXtProtiNX_O43272
Orphaneti419 Hyperprolinemia type 1
PharmGKBiPA33801

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG0186 Eukaryota
COG0506 LUCA
HOVERGENiHBG008262
InParanoidiO43272
KOiK00318
OrthoDBi912212at2759
PhylomeDBiO43272
TreeFamiTF313544

Enzyme and pathway databases

UniPathwayi
UPA00261;UER00373

BioCyciMetaCyc:HS01958-MONOMER
BRENDAi1.5.99.B2 2681
ReactomeiR-HSA-70688 Proline catabolism
SIGNORiO43272

Miscellaneous databases

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...
ChiTaRSi
PRODH human

The Gene Wiki collection of pages on human genes and proteins

More...
GeneWikii
Proline_oxidase

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...
GenomeRNAii
5625

Protein Ontology

More...
PROi
PR:O43272

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000100033 Expressed in 89 organ(s), highest expression level in right lung
ExpressionAtlasiO43272 baseline and differential
GenevisibleiO43272 HS

Family and domain databases

InterProiView protein in InterPro
IPR029041 FAD-linked_oxidoreductase-like
IPR002872 Proline_DH_dom
IPR015659 Proline_oxidase
PANTHERiPTHR13914 PTHR13914, 1 hit
PfamiView protein in Pfam
PF01619 Pro_dh, 1 hit
SUPFAMiSSF51730 SSF51730, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

More...
ProtoNeti
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiPROD_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: O43272
Secondary accession number(s): A6NF53
, O14680, Q0P507, Q147W8, Q504W1, Q59FI8, Q6NV86, Q9UF13
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 30, 2000
Last sequence update: April 5, 2011
Last modified: January 16, 2019
This is version 173 of the entry and version 3 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human chromosome 22
    Human chromosome 22: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PATHWAY comments
    Index of metabolic and biosynthesis pathways
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again