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PMPCA
Substrate recognition and binding subunit of the essential mitochondrial processing protease (MPP), which is required for maturation of the majority of mitochondrial precursor proteins. Most MPP cleavage sites follow an arginine at position -2. Belongs to the peptidase M16 family. Ubiquitously expressed with highest expression in fetal tissues and adult brain, cerebellum and cerebellar vermis. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
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| Protein type: EC 3.4.24.64; Mitochondrial; Protease |
| Chromosomal Location of human Ortholog: 9q34.3 |
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Cellular Component:
mitochondrial inner membrane; mitochondrial processing peptidase complex; mitochondrion
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Molecular Function:
metal ion binding; metalloendopeptidase activity; protein binding
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Biological Process:
protein processing involved in protein targeting to mitochondrion
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Disease: Spinocerebellar Ataxia, Autosomal Recessive 2
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Reference #:
Q10713
(UniProtKB)
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Alt. Names/Synonyms: Alpha-MPP; cerebellar ataxia 1 (autosomal recessive); Cerebellar ataxia-1; CLA1; CPD3; FLJ26258; Inactive zinc metalloprotease alpha; inositol polyphosphate-5-phosphatase, 72 kD; INPP5E; KIAA0123; MAS2; MGC104197; mitochondrial matrix processing protease, alpha subunit; Mitochondrial-processing peptidase subunit alpha; MPPA; P-55; peptidase (mitochondrial processing) alpha; peptidase, mitochondrial processing alpha subunit; peptidase, mitochondrial processing subunit alpha; PMPCA; SCAR2
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Gene Symbols: PMPCA
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Molecular weight:
58,253 Da
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Basal Isoelectric point:
6.45
Predict pI for various phosphorylation states
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