Clpx caseinolytic mitochondrial matrix peptidase chaperone subunit [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Clpxprovided by MGI
Official Full Name: caseinolytic mitochondrial matrix peptidase chaperone subunitprovided by MGI
Primary source: MGI:MGI:1346017
See related: Ensembl:ENSMUSG00000015357 AllianceGenome:MGI:1346017
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: E330029I21
Summary: Enables ATP binding activity and ATP hydrolysis activity. Predicted to be involved in ATP metabolic process and proteolysis involved in protein catabolic process. Located in mitochondrion. Is expressed in several structures, including alimentary system; brain; early conceptus; genitourinary system; and hemolymphoid system. Human ortholog(s) of this gene implicated in erythropoietic protoporphyria. Orthologous to human CLPX (caseinolytic mitochondrial matrix peptidase chaperone subunit X). [provided by Alliance of Genome Resources, Dec 2024]
Expression: Ubiquitous expression in liver E18 (RPKM 23.0), testis adult (RPKM 18.8) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 9 C; 9 35.22 cM

Exon count:: 14

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	9	NC_000075.7 (65201533..65237940)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	9	NC_000075.6 (65294251..65330658)

Chromosome 9 - NC_000075.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

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.
ClpP/ClpX deficiency impairs mitochondrial functions and mTORC1 signaling during spermatogenesis.
Title: ClpP/ClpX deficiency impairs mitochondrial functions and mTORC1 signaling during spermatogenesis.
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 ubiquitous mitochondrial protein unfoldase CLPX regulates erythroid heme synthesis by control of iron utilization and heme synthesis enzyme activation and turnover.
Title: The ubiquitous mitochondrial protein unfoldase CLPX regulates erythroid heme synthesis by control of iron utilization and heme synthesis enzyme activation and turnover.
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.
results illustrate that ClpX overexpression is a good and simple model to study the underlying mechanisms of the UPRmt in mammalian cells.
Title: ClpX stimulates the mitochondrial unfolded protein response (UPRmt) in mammalian cells.
Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors.
Title: Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors.

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Variation

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Alleles

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

Endonuclease-mediated (1)
Targeted (2)

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

AU014732 (e-PCR)
- UniSTS:208395

BB208476 (e-PCR)
- UniSTS:208398

RH126994 (e-PCR)
- UniSTS:167610

Clpx (e-PCR), detects polymorphism
- UniSTS:186342

Clpx (e-PCR), detects polymorphism
- UniSTS:186343

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables ATP binding	IBA Inferred from Biological aspect of Ancestor more info
enables ATP binding	IDA Inferred from Direct Assay more info	PubMed
enables ATP hydrolysis activity	IBA Inferred from Biological aspect of Ancestor more info
enables ATP hydrolysis activity	IDA Inferred from Direct Assay more info	PubMed
enables ATP hydrolysis activity	ISO Inferred from Sequence Orthology more info
contributes_to ATP-dependent peptidase activity	ISO Inferred from Sequence Orthology more info
contributes_to ATP-dependent peptidase activity	ISS Inferred from Sequence or Structural Similarity more info
enables ATP-dependent protein folding chaperone	IEA Inferred from Electronic Annotation more info
enables peptidase activator activity	IEA Inferred from Electronic Annotation more info
enables peptidase activator activity	ISO Inferred from Sequence Orthology more info
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables protein dimerization activity	IEA Inferred from Electronic Annotation more info
enables unfolded protein binding	IEA Inferred from Electronic Annotation more info
enables zinc ion binding	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
involved_in ATP metabolic process	IEA Inferred from Electronic Annotation more info
involved_in ATP metabolic process	ISO Inferred from Sequence Orthology more info
involved_in protein folding	IEA Inferred from Electronic Annotation more info
involved_in proteolysis	ISO Inferred from Sequence Orthology more info
involved_in proteolysis involved in protein catabolic process	IBA Inferred from Biological aspect of Ancestor 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
located_in cytosol	IEA Inferred from Electronic Annotation more info
located_in cytosol	ISO Inferred from Sequence Orthology 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
part_of mitochondrial endopeptidase Clp complex	IEA Inferred from Electronic Annotation more info
part_of mitochondrial endopeptidase Clp complex	ISO Inferred from Sequence Orthology more info
located_in mitochondrial inner membrane	HDA more info	PubMed
is_active_in mitochondrial matrix	IBA Inferred from Biological aspect of Ancestor 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 mitochondrial nucleoid	IEA Inferred from Electronic Annotation more info
located_in mitochondrial nucleoid	ISO Inferred from Sequence Orthology 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
located_in nucleoplasm	IEA Inferred from Electronic Annotation more info
located_in nucleoplasm	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial

Names: caseinolytic peptidase X; caseinolytic protease X

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_001044389.2 → NP_001037854.1 ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial isoform 2

See identical proteins and their annotated locations for NP_001037854.1

Status: VALIDATED

Description

Transcript Variant: This variant (2) lacks an alternate in-frame exon, compared to variant 1. It encodes isoform 2, which is shorter than isoform 1.

Source sequence(s)

AC110235, AK087848, AV237409, BC061153

Consensus CDS

CCDS40669.1

UniProtKB/TrEMBL

Q6P8N8

Related

ENSMUSP00000109455.2, ENSMUST00000113824.8

Conserved Domains (4) summary

smart01086
Location:496 → 590

ClpB_D2-small; C-terminal, D2-small domain, of ClpB protein

TIGR00382
Location:103 → 587

clpX; endopeptidase Clp ATP-binding regulatory subunit (clpX)

pfam07724
Location:273 → 489

AAA_2; AAA domain (Cdc48 subfamily)

cl02609
Location:105 → 133

Zn-ribbon; C-terminal zinc ribbon domain of RNA polymerase intrinsic transcript cleavage subunit
NM_011802.3 → NP_035932.2 ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial isoform 1

See identical proteins and their annotated locations for NP_035932.2

Status: VALIDATED

Description

Transcript Variant: This variant (1) represents the longer transcript and encodes the longer isoform (1).

Source sequence(s)

AC110235, AK080339, AK087848, AV237409

Consensus CDS

CCDS23288.1

UniProtKB/Swiss-Prot

E9QLZ8, Q9JHS4, Q9WVD1

Related

ENSMUSP00000015501.5, ENSMUST00000015501.11

Conserved Domains (4) summary

smart01086
Location:510 → 604

ClpB_D2-small; C-terminal, D2-small domain, of ClpB protein

TIGR00382
Location:103 → 601

clpX; endopeptidase Clp ATP-binding regulatory subunit (clpX)

pfam07724
Location:287 → 503

AAA_2; AAA domain (Cdc48 subfamily)

cl02609
Location:105 → 133

Zn-ribbon; C-terminal zinc ribbon domain of RNA polymerase intrinsic transcript cleavage subunit