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Peptidase (mitochondrial processing) beta
Symbols PMPCB ; Beta-MPP; MPP11; MPPB; MPPP52; P-52
External IDs OMIM603131 MGI1920328 HomoloGene3160 GeneCards: PMPCB Gene
EC number
RNA expression pattern
PBB GE PMPCB 201682 at tn.png
More reference expression data
Species Human Mouse
Entrez 9512 73078
Ensembl ENSG00000105819 ENSMUSG00000029017
UniProt O75439 Q9CXT8
RefSeq (mRNA) NM_004279 NM_028431
RefSeq (protein) NP_004270 NP_082707
Location (UCSC) Chr 7:
103.3 – 103.33 Mb
Chr 5:
21.74 – 21.76 Mb
PubMed search [1] [2]

Mitochondrial-processing peptidase subunit beta is an enzyme that in humans is encoded by the PMPCB gene.[1][2]

This gene is a member of the peptidase M16 family and encodes a protein with a zinc-binding motif. This protein is located in the mitochondrial matrix and catalyzes the cleavage of the leader peptides of precursor proteins newly imported into the mitochondria, though it only functions as part of a heterodimeric complex.[2]


PMPCB has been shown to interact with Frataxin.[3]


  1. ^ Mao M, Fu G, Wu JS, Zhang QH, Zhou J, Kan LX, Huang QH, He KL, Gu BW, Han ZG, Shen Y, Gu J, Yu YP, Xu SH, Wang YX, Chen SJ, Chen Z (Aug 1998). "Identification of genes expressed in human CD34(+) hematopoietic stem/progenitor cells by expressed sequence tags and efficient full-length cDNA cloning". Proc Natl Acad Sci U S A 95 (14): 8175–80. doi:10.1073/pnas.95.14.8175. PMC 20949. PMID 9653160. 
  2. ^ a b "Entrez Gene: PMPCB peptidase (mitochondrial processing) beta". 
  3. ^ Koutnikova, H; Campuzano V; Koenig M (Sep 1998). "Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase". Hum. Mol. Genet. (ENGLAND) 7 (9): 1485–9. doi:10.1093/hmg/7.9.1485. ISSN 0964-6906. PMID 9700204. 

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