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Details on Person UniProt:Q10713 PMPCA
| Class:Id | ReferenceGeneProduct:59533 |
|---|---|
| _chainChangeLog | transit peptide:1-33 added on Fri February 6 2015;chain:34-525 added on Fri February 6 2015 |
| _displayName | UniProt:Q10713 PMPCA |
| _timestamp | 2024-11-03 20:01:53 |
| chain | transit peptide:1-33 chain:34-525 |
| checksum | 2751E7FCDC864E3F |
| comment | FUNCTION Substrate recognition and binding subunit of the essential mitochondrial processing protease (MPP), which cleaves the mitochondrial sequence off newly imported precursors proteins.SUBUNIT Heterodimer of PMPCA (alpha) and PMPCB (beta) subunits, forming the mitochondrial processing protease (MPP) in which PMPCA is involved in substrate recognition and binding and PMPCB is the catalytic subunit.INTERACTION Ubiquitously expressed with highest expression in fetal tissues and adult brain, cerebellum and cerebellar vermis.MASS SPECTROMETRY The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the peptidase M16 family.CAUTION Does not seem to have protease activity as it lacks the zinc-binding site. |
| description | recommendedName: Mitochondrial-processing peptidase subunit alpha alternativeName: Alpha-MPP alternativeName: fullName evidence="8"Inactive zinc metalloprotease alpha alternativeName: P-55 |
| geneName | PMPCA INPP5E KIAA0123 MPPA |
| identifier | Q10713 |
| isSequenceChanged | FALSE |
| keyword | Acetylation Alternative splicing Disease variant Intellectual disability Membrane Mitochondrion Mitochondrion inner membrane Neurodegeneration Proteomics identification Reference proteome Transit peptide |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 |
| name | PMPCA |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8999004] ENSEMBL:ENSG00000165688 PMPCA [Homo sapiens] |
| secondaryIdentifier | MPPA_HUMAN B4DKL3 E7ET61 Q16639 Q5SXM9 Q8N513 |
| sequenceLength | 525 |
| species | [Species:48887] Homo sapiens |
| (isoformParent) | [ReferenceIsoform:8972942] UniProt:Q10713-1 PMPCA [Homo sapiens] [ReferenceIsoform:8972943] UniProt:Q10713-2 PMPCA [Homo sapiens] |
| (referenceEntity) | [EntityWithAccessionedSequence:1299450] PMPCA [mitochondrial matrix] [Homo sapiens] |
| [Change default viewing format] | |
No pathways have been reviewed or authored by UniProt:Q10713 PMPCA (59533)
