Clpp caseinolytic mitochondrial matrix peptidase proteolytic subunit [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Clppprovided by MGI
Official Full Name: caseinolytic mitochondrial matrix peptidase proteolytic subunitprovided by MGI
Primary source: MGI:MGI:1858213
See related: Ensembl:ENSMUSG00000002660 AllianceGenome:MGI:1858213
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: D17Wsu160e
Summary: Predicted to enable ATPase binding activity; identical protein binding activity; and peptidase activity. Predicted to be involved in membrane protein proteolysis and protein quality control for misfolded or incompletely synthesized proteins. Located in mitochondrion. Is expressed in several structures, including early conceptus; heart; liver; pancreas; and placenta. Used to study Perrault syndrome. Human ortholog(s) of this gene implicated in Perrault syndrome. Orthologous to human CLPP (caseinolytic mitochondrial matrix peptidase proteolytic subunit). [provided by Alliance of Genome Resources, Nov 2024]
Expression: Ubiquitous expression in adrenal adult (RPKM 124.9), ovary adult (RPKM 93.5) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 17 D; 17 29.61 cM

Exon count:: 6

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	17	NC_000083.7 (57297264..57303371)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	17	NC_000083.6 (56990264..56996371)

Chromosome 17 - NC_000083.7 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?

Knockout Mouse Studies Show That Mitochondrial CLPP Peptidase and CLPX Unfoldase Act in Matrix Condensates near IMM, as Fast Stress Response in Protein Assemblies for Transcript Processing, Translation, and Heme Production.
Title: Knockout Mouse Studies Show That Mitochondrial CLPP Peptidase and CLPX Unfoldase Act in Matrix Condensates near IMM, as Fast Stress Response in Protein Assemblies for Transcript Processing, Translation, and Heme Production.
CLPP-Null Eukaryotes with Excess Heme Biosynthesis Show Reduced L-arginine Levels, Probably via CLPX-Mediated OAT Activation.
Title: CLPP-Null Eukaryotes with Excess Heme Biosynthesis Show Reduced L-arginine Levels, Probably via CLPX-Mediated OAT Activation.
Mitochondrial Unfolded Protein Response Gene Clpp Is Required for Oocyte Function and Female Fertility.
Title: Mitochondrial Unfolded Protein Response Gene Clpp Is Required for Oocyte Function and Female Fertility.
Translation Fidelity and Respiration Deficits in CLPP-Deficient Tissues: Mechanistic Insights from Mitochondrial Complexome Profiling.
Title: Translation Fidelity and Respiration Deficits in CLPP-Deficient Tissues: Mechanistic Insights from Mitochondrial Complexome Profiling.
ClpP/ClpX deficiency impairs mitochondrial functions and mTORC1 signaling during spermatogenesis.
Title: ClpP/ClpX deficiency impairs mitochondrial functions and mTORC1 signaling during spermatogenesis.
CLPP Depletion Causes Diplotene Arrest; Underlying Testis Mitochondrial Dysfunction Occurs with Accumulation of Perrault Proteins ERAL1, PEO1, and HARS2.
Title: CLPP Depletion Causes Diplotene Arrest; Underlying Testis Mitochondrial Dysfunction Occurs with Accumulation of Perrault Proteins ERAL1, PEO1, and HARS2.
KPNB1 modulates the Machado-Joseph disease protein ataxin-3 through activation of the mitochondrial protease CLPP.
Title: KPNB1 modulates the Machado-Joseph disease protein ataxin-3 through activation of the mitochondrial protease CLPP.
CLPP deficiency ameliorates neurodegeneration caused by impaired mitochondrial protein synthesis.
Title: CLPP deficiency ameliorates neurodegeneration caused by impaired mitochondrial protein synthesis.
Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA.
Title: Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA.
The Mouse Heart Mitochondria N Terminome Provides Insights into ClpXP-Mediated Proteolysis.
Title: The Mouse Heart Mitochondria N Terminome Provides Insights into ClpXP-Mediated Proteolysis.

Submit: New GeneRIF Correction See all GeneRIFs (19)

Variation

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Alleles

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

Endonuclease-mediated (4)
Gene trapped (3)
Targeted (6) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

Clpp (e-PCR), detects polymorphism
- UniSTS:164891

Clpp (e-PCR), detects polymorphism
- UniSTS:164892

D17Wsu160e (e-PCR), detects polymorphism
- UniSTS:142172

RH136298 (e-PCR)
- UniSTS:210290

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables ATP-dependent peptidase activity	IBA Inferred from Biological aspect of Ancestor more info
enables ATP-dependent peptidase activity	IEA Inferred from Electronic Annotation more info
enables ATPase binding	IBA Inferred from Biological aspect of Ancestor more info
enables endopeptidase activity	ISO Inferred from Sequence Orthology more info
enables identical protein binding	ISO Inferred from Sequence Orthology more info
enables peptidase activity	ISA Inferred from Sequence Alignment more info	PubMed
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables serine-type endopeptidase activity	IBA Inferred from Biological aspect of Ancestor more info
enables serine-type endopeptidase activity	ISO Inferred from Sequence Orthology more info
enables serine-type endopeptidase activity	ISS Inferred from Sequence or Structural Similarity more info

Process	Evidence Code	Pubs
involved_in membrane protein proteolysis	IEA Inferred from Electronic Annotation more info
involved_in membrane protein proteolysis	ISO Inferred from Sequence Orthology more info
involved_in protein quality control for misfolded or incompletely synthesized proteins	IBA Inferred from Biological aspect of Ancestor more info
acts_upstream_of_or_within protein quality control for misfolded or incompletely synthesized proteins	ISA Inferred from Sequence Alignment more info	PubMed
involved_in proteolysis	ISO Inferred from Sequence Orthology more info
involved_in proteolysis involved in protein catabolic process	ISO Inferred from Sequence Orthology more info
involved_in proteolysis involved in protein catabolic process	ISS Inferred from Sequence or Structural Similarity more info

Component	Evidence Code	Pubs
part_of endopeptidase Clp complex	IBA Inferred from Biological aspect of Ancestor more info
part_of endopeptidase Clp complex	ISO Inferred from Sequence Orthology more info
part_of endopeptidase Clp complex	ISS Inferred from Sequence or Structural Similarity more info
located_in mitochondrial matrix	ISO Inferred from Sequence Orthology more info
located_in mitochondrial matrix	ISS Inferred from Sequence or Structural Similarity more info
located_in mitochondrion	HDA more info	PubMed
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: ATP-dependent Clp protease proteolytic subunit, mitochondrial

Names: ClpP caseinolytic peptidase, ATP-dependent, proteolytic subunit; caseinolytic peptidase, ATP-dependent, proteolytic subunit homolog; caseinolytic protease, ATP-dependent, proteolytic subunit homolog; endopeptidase Clp; putative ATP-dependent Clp protease proteolytic subunit, mitochondrial

NP_059089.1

EC 3.4.21.92

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_017393.2 → NP_059089.1 ATP-dependent Clp protease proteolytic subunit, mitochondrial precursor

See identical proteins and their annotated locations for NP_059089.1

Status: VALIDATED

Source sequence(s)

AC073683, AK145765, BY294002

Consensus CDS

CCDS28920.1

UniProtKB/Swiss-Prot

O88696, Q3TI13

UniProtKB/TrEMBL

Q3UGW4

Related

ENSMUSP00000002735.8, ENSMUST00000002735.9

Conserved Domains (1) summary

PRK00277
Location:53 → 245

clpP; ATP-dependent Clp protease proteolytic subunit; Reviewed