Search by BoMiProt ID - Bomi7157

Primary Information

BoMiProt ID Bomi7157
Protein Name Mitochondrial-processing peptidase subunit alpha/Alpha-MPP/Inactive zinc metalloprotease alpha
Organism Bos taurus
Uniprot IDQ0P5M8
Milk FractionWhey
Ref Sequence ID NP_001070432.1
Aminoacid Length 525
Molecular Weight 58135
FASTA Sequence Download
Gene Name PMPCA
Gene ID 767847
Protein Existence Status reviewed

Secondary Information

Protein Function the alpha subunit of mitochondrial processing peptidase,which is a heterodimeric enzyme responsible for the cleavage of nuclear-encoded mitochondrial precursor proteins after import in the mitochondria.Also its responsible for the maturation of the vast majority of nuclear-encoded mitochondrial proteins, which is necessary for life at the cellular level.
Biochemical Properties α-MPP is uniquely involved in substrate recognition and binding. The α-MPP subunit can bind precursor proteins in the absence of β-MPP with the same efficiency as the MPP heterodimer but does not cleave the substrate .
PTMs Acetylation,Proteolyitc digestion
Site(s) of PTM(s)

N-glycosylation, O-glycosylation,
Predicted Disorder Regions NA
DisProt Annotation
TM Helix Prediction No TM helices
Bibliography 1.Jobling RK, Assoum M, Gakh O, Blaser S, Raiman JA, Mignot C, Roze E, Dürr A, Brice A, Lévy N, Prasad C, Paton T, Paterson AD, Roslin NM, Marshall CR, Desvignes JP, Roëckel-Trevisiol N, Scherer SW, Rouleau GA, Mégarbané A, Isaya G, Delague V, Yoon G. PMPCA mutations cause abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia. Brain. 2015 Jun;138(Pt 6):1505-17. doi: 10.1093/brain/awv057. Epub 2015 Mar 25. PMID: 25808372; PMCID: PMC4542620.