Pmpcb peptidase (mitochondrial processing) beta [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Pmpcbprovided by MGI
Official Full Name: peptidase (mitochondrial processing) betaprovided by MGI
Primary source: MGI:MGI:1920328
See related: Ensembl:ENSMUSG00000029017 AllianceGenome:MGI:1920328
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: MPPB; MPP11; MPPP52; 3110004O18Rik
Summary: Predicted to enable metalloendopeptidase activity. Predicted to contribute to metallopeptidase activity. Predicted to be involved in protein processing involved in protein targeting to mitochondrion. Located in mitochondrial inner membrane. Is expressed in several structures, including extraembryonic component; gallbladder; genitourinary system; gut gland; and nervous system. Human ortholog(s) of this gene implicated in multiple mitochondrial dysfunctions syndrome 6. Orthologous to human PMPCB (peptidase, mitochondrial processing subunit beta). [provided by Alliance of Genome Resources, Nov 2024]
Expression: Ubiquitous expression in kidney adult (RPKM 46.3), heart adult (RPKM 42.1) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 5 A3; 5 9.97 cM

Exon count:: 13

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	5	NC_000071.7 (21942158..21962150)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	5	NC_000071.6 (21737160..21757152)

Chromosome 5 - NC_000071.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

OxLDL triggers retrograde translocation of arginase2 in aortic endothelial cells via ROCK and mitochondrial processing peptidase.
Title: OxLDL triggers retrograde translocation of arginase2 in aortic endothelial cells via ROCK and mitochondrial processing peptidase.
Data show that Afg3l1 or Afg3l2 are required for maturation of newly imported paraplegin subunits after their cleavage by MPP.
Title: Autocatalytic processing of m-AAA protease subunits in mitochondria.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (2)
Targeted (2)
Transgenic (1)

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables endopeptidase activity	ISO Inferred from Sequence Orthology more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables metalloendopeptidase activity	IBA Inferred from Biological aspect of Ancestor more info
enables metalloendopeptidase activity	IEA Inferred from Electronic Annotation more info
enables metalloendopeptidase activity	ISO Inferred from Sequence Orthology more info
contributes_to metallopeptidase activity	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
involved_in protein processing	ISO Inferred from Sequence Orthology more info
involved_in protein processing involved in protein targeting to mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
involved_in protein processing involved in protein targeting to mitochondrion	IEA Inferred from Electronic Annotation more info
involved_in protein processing involved in protein targeting to mitochondrion	ISO Inferred from Sequence Orthology more info

Component	Evidence Code	Pubs
located_in mitochondrial inner membrane	HDA more info	PubMed
located_in mitochondrial matrix	IEA Inferred from Electronic Annotation more info
part_of mitochondrial processing peptidase complex	ISO Inferred from Sequence Orthology more info
located_in mitochondrion	HDA more info	PubMed
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: mitochondrial-processing peptidase subunit beta

Names: P-52; beta-MPP

NP_001404726.1

EC 3.4.24.64

NP_082707.1

EC 3.4.24.64

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_001417797.1 → NP_001404726.1 mitochondrial-processing peptidase subunit beta isoform 2

Status: VALIDATED

Source sequence(s)

AC125313, AC125343
NM_028431.2 → NP_082707.1 mitochondrial-processing peptidase subunit beta isoform 1 precursor

See identical proteins and their annotated locations for NP_082707.1

Status: VALIDATED

Source sequence(s)

AK013995

Consensus CDS

CCDS19106.1

UniProtKB/Swiss-Prot

Q9CXT8

UniProtKB/TrEMBL

Q3TET5

Related

ENSMUSP00000030882.6, ENSMUST00000030882.12

Conserved Domains (1) summary

COG0612
Location:46 → 478

PqqL; Predicted Zn-dependent peptidase [General function prediction only]