1. Gene
  2. CLPX - caseinolytic mitochondrial matrix peptidase chaperone subunit X Gene

CLPX - caseinolytic mitochondrial matrix peptidase chaperone subunit X Gene

中文名称:酪蛋白分解线粒体基质肽酶伴侣亚基 X

种属: Homo sapiens

同用名: EPP2

基因 ID: 10845 | 基因类型: protein coding

关于 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.

功能概要

由该基因编码的蛋白质是在线粒体中发现的蛋白酶的一部分。这种蛋白酶是 ATP 依赖性的,靶向特定蛋白质进行降解。该蛋白酶由 ClpP 催化亚基的两个七聚体环组成,夹在由该基因编码的伴侣亚基的两个六聚体环之间。目标蛋白被该蛋白解开,然后传递给 ClpP 亚基进行降解。已发现该基因的两种转录变体,一种是蛋白质编码,另一种是非蛋白质编码。[RefSeq 提供,2015 年 11 月]

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 基因产物(1)

mRNA Protein Name
NM_006660.5 NP_006651.2 ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial precursor
基因本体论
  • 分子功能
  • 生物过程
  • 细胞组分
分子功能 GO 注释 逻辑证据 参考文献 来源
enables ATP hydrolysis activity IDA
IDA: 通过直接分析推断
22710082 GOA
contributes to ATP-dependent peptidase activity IDA
IDA: 通过直接分析推断
16115876 GOA
enables peptidase activator activity IDA
IDA: 通过直接分析推断
11923310 GOA
enables protein binding IPI
IPI: 通过物理相互作用推断
11923310 GOA
生物过程 GO 注释 逻辑证据 参考文献 来源
involved in ATP metabolic process IDA
IDA: 通过直接分析推断
22710082 GOA
involved in proteolysis IDA
IDA: 通过直接分析推断
15522782 GOA
involved in proteolysis involved in protein catabolic process IDA
IDA: 通过直接分析推断
16115876 GOA
细胞组分 GO 注释 逻辑证据 参考文献 来源
part of endopeptidase Clp complex IDA
IDA: 通过直接分析推断
11923310 GOA
part of endopeptidase Clp complex IPI
IPI: 通过物理相互作用推断
15522782 GOA
part of mitochondrial endopeptidase Clp complex IDA
IDA: 通过直接分析推断
16115876 GOA
located in mitochondrial matrix IDA
IDA: 通过直接分析推断
10525407 GOA
located in mitochondrial nucleoid IDA
IDA: 通过直接分析推断
18063578 GOA
located in mitochondrion IDA
IDA: 通过直接分析推断
11003706 GOA
EXP:通过实验结果推断 IDA:通过直接分析推断 IPI:通过物理相互作用推断 IMP:通过突变表型推断 IGI:通过遗传相互作用推断 IEP:通过表达模式推断

CLPX 蛋白结构

AAA_2

AAA_2: AAA domain (Cdc48 subfamily) (285 - 502)

ClpB_D2-small

ClpB_D2-small: C-terminal, D2-small domain, of ClpB protein (509 - 581)

  • 0
  • 100
  • 200
  • 300
  • 400
  • 500
  • 633 a.a.
蛋白主名 其他名称

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

ClpX caseinolytic peptidase X homolog

CLPX 抗体

目录号 产品名 应用 反应物种
HY-P83069 CLPX Antibody (YA2814) WB, ICC/IF Human, Mouse, Rat, Hamster

关联疾病

疾病名称 别名
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

疾病名称 别名
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

直系同源

种属 基因名 来源 基因 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