CLPX - caseinolytic mitochondrial matrix peptidase chaperone subunit X Gene

Also Known as EPP2

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 10845

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	References	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	References	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	References	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
600
633 a.a.

Protein Preferred Names	Protein Names
ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial	ClpX caseinolytic peptidase X homolog

CLPX Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P83069	CLPX Antibody (YA2814)	WB, ICC/IF	Human, Mouse, Rat, Hamster
HY-P83069A	CLPX Antibody (YA2814)(PBS only)	WB, ICC/IF	Human, Mouse, Rat, Hamster

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

Pre-clinical Phase

Bioactive Molecules (4)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS02835	CLPX Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-178912	Anti-MRSA agent 39		/	Unkown
HY-RS22125	Clpx Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS28647	Clpx Rat Pre-designed siRNA Set A	Pre-designed Set	/	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
Others	CLPX	NCBI