Entity Details

Primary name MPPB_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionO75439
EntryNameMPPB_HUMAN
FullNameMitochondrial-processing peptidase subunit beta
TaxID9606
Evidenceevidence at protein level
Length489
SequenceStatuscomplete
DateCreated1998-12-15
DateModified2021-06-02

Ontological Relatives

GenesPMPCB

GO terms

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GOName
GO:0004222 metalloendopeptidase activity
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0006627 protein processing involved in protein targeting to mitochondrion
GO:0006851 mitochondrial calcium ion transmembrane transport
GO:0017087 mitochondrial processing peptidase complex
GO:0046872 metal ion binding

Subcellular Location

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Subcellular Location
Mitochondrion matrix

Domains

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DomainNameCategoryType
IPR001431 Peptidase M16, zinc-binding siteSiteBinding site
IPR007863 Peptidase M16, C-terminalDomainDomain
IPR011249 Metalloenzyme, LuxS/M16 peptidase-likeFamilyHomologous superfamily
IPR011765 Peptidase M16, N-terminalDomainDomain
IPR037718 Mitochondrial processing peptidase subunit betaFamilyFamily

Diseases

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Disease IDSourceNameDescription
617954 OMIMMultiple mitochondrial dysfunctions syndrome 6 (MMDS6)An autosomal recessive, neurodegenerative disorder characterized by basal ganglia lesions, cerebellar atrophy, and neurologic regression in the first year of life. Common features include truncal hypotonia, lack of independent ambulation, poor speech, intellectual disability, and motor abnormalities, such as ataxia, dystonia, and spasticity. The disease is caused by variants affecting the gene represented in this entry.

Interactions

6 interactions