CLPX - caseinolytic mitochondrial matrix peptidase chaperone subunit X Gene

Homo sapiens

Also known as EPP2

Gene ID: 10845 | Gene type: protein coding

About CLPX

Cytogenetic location: 15q22.31 Genomic coordinates (GRCh38): 15:65,148,219-65,185,342 (from NCBI)

This gene has 6 transcripts (splice variants), 239 orthologues and is associated with 1 phenotype. Ubiquitous expression in liver (RPKM 13.6), testis (RPKM 11.0) and 25 other tissues.

Summary

The protein encoded by this gene is part of a Protease found in mitochondria. This Protease is ATP-dependent and targets specific proteins for degradation. The Protease consists of two heptameric rings of the ClpP catalytic subunit sandwiched between two hexameric rings of the chaperone subunit encoded by this gene. Targeted proteins are unwound by this protein and then passed on to the ClpP subunit for degradation. Two transcript variants, one protein-coding and the other non-protein coding, have been found for this gene. [provided by RefSeq, Nov 2015]

CLPX Products(1)

mRNA	Protein	Name
NM_006660.5	NP_006651.2	ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	Source
enables ATP hydrolysis activity	IDA IDA: Inferred from direct assay	22710082	GOA
contributes to ATP-dependent peptidase activity	IDA IDA: Inferred from direct assay	16115876	GOA
enables peptidase activator activity	IDA IDA: Inferred from direct assay	11923310	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	11923310	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in ATP metabolic process	IDA IDA: Inferred from direct assay	22710082	GOA
involved in proteolysis	IDA IDA: Inferred from direct assay	15522782	GOA
involved in proteolysis involved in protein catabolic process	IDA IDA: Inferred from direct assay	16115876	GOA

Cellular Component GO Annotation	Evidence	Reference	Source
part of endopeptidase Clp complex	IDA IDA: Inferred from direct assay	11923310	GOA
part of endopeptidase Clp complex	IPI IPI: Inferred from physical interaction	15522782	GOA
part of mitochondrial endopeptidase Clp complex	IDA IDA: Inferred from direct assay	16115876	GOA
located in mitochondrial matrix	IDA IDA: Inferred from direct assay	10525407	GOA
located in mitochondrial nucleoid	IDA IDA: Inferred from direct assay	18063578	GOA
located in mitochondrion	IDA IDA: Inferred from direct assay	11003706	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

CLPX Protein Structure

AAA_2

ClpB_D2-small

0
100
200
300
400
500
633 a.a.

Protein Preferred Names

Protein Names

ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial

ClpX caseinolytic peptidase X homolog

Related Diseases

Diseases

Alias

Protoporphyria, Erythropoietic, 2

EPP2

Tick-Borne Relapsing Fever

Relapsing Fever, Tick-Borne	Relapsing Fever Due To Any Borrelia Species Other Than Borrelia Recurrentis
African Tick-Borne Fever

Porphyria

Hematoporphyria	Porphyrias
Disorder Of Porphyrin And Hem Metabolism	Disorder Of Porphyrin Metabolism
Porphyrinopathy	Porphyrin Disorder
Disorder Of Porphyrin And Heme Metabolism	Disorders Of Porphyrin Metabolism

Acrodermatitis Chronica Atrophicans

Acrodermatitis Atrophicans Chronica	Herxheimer Disease
Primary Diffuse Atrophy

Spinocerebellar Ataxia 28

Spinocerebellar Ataxia Type 28	SCA28
Ataxia, Spinocerebellar, Type 28

Relapsing Fever

Febris Recurrens	Novy Febris Recurrens
Novy Relapsing Fever

Codas Syndrome

Cerebral, Ocular, Dental, Auricular, And Skeletal Anomalies Syndrome	Cerebral, Ocular, Dental, Auricular, And Skeletal Syndrome
Cerebro-Oculo-Dento-Auriculo-Skeletal Syndrome	Cerebrooculodentoauriculoskeletal Syndrome
CODASS	Cerebral, Ocular, Dental, Auricular, Skeletal Syndrome

Protoporphyria, Erythropoietic, 1

Erythropoietic Protoporphyria	Epp
Ferrochelatase Deficiency	Protoporphyria, Erythropoietic
Erythrohepatic Protoporphyria	Heme Synthetase Deficiency
Autosomal Erythropoietic Protoporphyria	EPP1
Protoporphyria	Protoporphyria Erythropoietic

Spastic Ataxia 5

Perrault Syndrome

Gonadal Dysgenesis, Xx Type, With Deafness	Ovarian Dysgenesis With Sensorineural Deafness
Gonadal Dysgenesis, Xx Type	Gonadal Dysgenesis With Auditory Dysfunction, Autosomal Recessive Inheritance
Gonadal Dysgenesis With Sensorineural Deafness, Autosomal Recessive Inheritance	Xx Gonodal Dysgenesis-Deafness Syndrome
Xx Gonodal Dysgenesis-Hearing Loss Syndrome	Gonadal Dysgenesis Xx Type Deafness

Cutaneous Porphyria

Porphyria, Erythropoietic

Erythropoietic Porphyria

Acute Porphyria

Porphyrias, Hepatic	Hepatic Porphyria
Porphyria Hepatic	Acute Intermittent Porphyria

Porphyria Cutanea Tarda

Hepatoerythropoietic Porphyria	HEP
Uroporphyrinogen Decarboxylase Deficiency	Pct
Pct, Type Ii	Porphyria, Hepatocutaneous Type
Urod Deficiency	Porphyria, Hepatoerythropoietic
Porphyria Cutanea Tarda, Susceptibility To	Familial Porphyria Cutanea Tarda
Porphyria Cutanea Tarda, Type Ii	Pct, 'Familial' Type
Porphyria, Hepatic	FPCT
Pct Type Ii	Porphyria Cutanea Tarda Type Ii
Porphyria Hepatocutaneous Type	Heterozygous Uroporphyrinogen Decarboxylase Deficiency
Urod - [Uroporphyrinogen Decarboxylase] Deficiency	Pct - [Porphyria Cutanea Tarda]

Granulomatous Disease, Chronic, Autosomal Recessive, 2

Chronic Granulomatous Disease Due To Deficiency Of Ncf-2	CGD2
Ncf2 Deficiency	P67-Phox Deficiency
Autosomal Recessive Chronic Granulomatous Disease Cytochrome B-Positive Type Ii	Granulomatous Disease, Chronic, Autosomal Recessive, Cytochrome B-Positive, Type 2
Granulomatous Disease, Chronic, Autosomal Recessive, Cytochrome B-Positive, Type Ii	Cgd, Autosomal Recessive Cytochrome B-Positive, Type Ii
Granulomatous Disease, Chronic, Due To Ncf2 Deficiency	Neutrophil Cytosol Factor 2 Deficiency
Chronic Granulomatous Disease 2, Autosomal Recessive	Autosomal Recessive Chronic Granulomatous Disease 2
Cdg2	Deficiency Of Ncf2
Deficiency Of P67-Phox	Chronic Granulomatous Disease Autosomal Recessive Cytochrome B-Positive Type Ii
Deficiency Of Neutrophil Cytosol Factor 2	Avellino Corneal Dystrophy

Coproporphyria, Hereditary

Hereditary Coproporphyria	Coproporphyria
Coproporphyrinogen Oxidase Deficiency	HCP
Cpo Deficiency	Cpox Deficiency
Cpx Deficiency	Hereditary Coproporphyria Porphyria
Cpro Deficiency	Coproporphyria Hereditary
Porphyria Hepatica Ii	Porphyria Hepatica Coproporphyria
Porphyria, Hereditary Coproporphyria	Harderoporphyria

Variegate Porphyria

Porphyria Variegata	Protoporphyrinogen Oxidase Deficiency
VP	Ppox Deficiency
Porphyria, South African Type	Porphyria Variegata, Susceptibility To
Protocoproporphyria	Porphyria Variegate
Porphyria South African Type	Pv
Porphyria, Variegate	Vp - [Variegate Porphyria]

Hereditary Spastic Paraplegia

Familial Spastic Paraplegia	Hereditary Spastic Paraparesis
Strumpell-Lorrain Disease	Familial Spastic Paraparesis
Hsp	Spg
Strümpell-Lorrain Disease	Spastic Paraplegia, Hereditary
French Settlement Disease	Strumpell-Lorrain Syndrome
Fsp	Spastic Paraplegia, Familial
Spastic Paraplegia Hereditary	Spastic Paraplegia 3, Autosomal Dominant
Spastic Paraparesis	Hereditary Spastic Paralysis
Familial Spastic Paralysis	Hereditary Spastic Ataxia

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS02835	CLPX Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Macaca mulatta	CLPX	VGNC	VGNC:84326
Bos taurus	CLPX	VGNC	VGNC:27462
Mus musculus	CLPX	MGD	MGI:1346017
Rattus norvegicus	CLPX	RGD	RGD:1304883
Felis catus	CLPX	VGNC	VGNC:60976
Canis familiaris	CLPX	VGNC	VGNC:39359

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