Protein basic information
| LiverAtlas Protein ID |
HuLPr29157 |
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Uniprot ID |
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Uniprot Acc |
P32322;Q6FHI4;Q96DI6; |
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Protein name |
Pyrroline-5-carboxylate reductase 1, mitochondrial |
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Comment |
FUNCTION:Housekeeping enzyme that catalyzes the last step in proline biosynthesis. Can utilize both NAD and NADP, but has higher affinity for NAD. Involved in the cellular response to oxidative stress.||CATALYTIC ACTIVITY:L-proline + NAD(P)(+) = 1-pyrroline-5- carboxylate + NAD(P)H.||ENZYME REGULATION:Subject to competitive inhibition by the reaction product proline. Subject to competitive inhibition by stearoyl coenzyme A.||BIOPHYSICOCHEMICAL PROPERTIES:Kinetic parameters: KM=0.151 mM for NAD(+); KM=3.06 mM for NADP(+);||PATHWAY:Amino-acid biosynthesis; L-proline biosynthesis; L- proline from L-glutamate 5-semialdehyde:step 1/1.||SUBUNIT:Homodecamer; composed of 5 homodimers.||SUBCELLULAR LOCATION:Mitochondrion.||DISEASE:Defects in PYCR1 are the cause of cutis laxa autosomal recessive type 2B (ARCL2B) [MIM:612940]. A multisystem disorder characterized by the appearance of premature aging, wrinkled and lax skin with reduced elasticity, joint laxity, craniofacial dysmorphic features, |
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Subcellular localization |
Mitochondrion. |
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Gene name |
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Related liver disease name |
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Protein sequence
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MSVGFIGAGQLAFALAKGFTAAGVLAAHKIMASSPDMDLA TVSALRKMGVKLTPHNKETVQHSDVLFLAVKPHIIPFIL DEIGADIEDRHIVVSCAAGVTISSIEKKLSAFRPAPRVI RCMTNTPVVVREGATVYATGTHAQVEDGRLMEQLLSSVG FCTEVEEDLIDAVTGLSGSGPAYAFTALDALADGGVKMG LPRRLAVRLGAQALLGAAKMLLHSEQHPGQLKDNVSSPG GATIHALHVLESGGFRSLLINAVEASCIRTRELQSMADQ EQVSPAAIKKTILDKVKLDSPAGTALSPSGHTKLLPRSLAPAGKD |
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Database cross reference |
RefSeq Protein accession:NP_008838
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Liver relevance
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HCC significant Proteins |
Yes/No |
Yes |
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Quality score |
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HLPP validation |
Yes/No |
Yes |
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Project name |
Chinese Liver; |
Ontology annotation
| GO-C |
GO:0005759;C:mitochondrial matrix;TAS:Reactome. |
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GO-F |
GO:0005488;F:binding;IEA:InterPro. GO:0004735;F:pyrroline-5-carboxylate reductase activity;IDA:UniProtKB. |
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GO-P |
GO:0034599;P:cellular response to oxidative stress;IMP:UniProtKB. GO:0055114;P:oxidation-reduction process;IEA:UniProtKB-KW. GO:0006561;P:proline biosynthetic process;IDA:UniProtKB. |
Post-translational modification
| LiverAtlas Protein ID |
MOD type1 |
Position1 |
Residue1 |
Source name1 |
source ID1 |
Source method |
HLPP validation1 (Yes/no) |
Quality score |
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HuLPr29157 |
PHOSPHORYLATION |
233 |
S |
PhosphoSitePlus |
HTP |
N |
 
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HuLPr29157 |
PHOSPHORYLATION |
301 |
S |
PhosphoSitePlus |
LTP |
N |
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HuLPr29157 |
PHOSPHORYLATION |
303 |
S |
PhosphoSitePlus |
HTP |
N |
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