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UniProtKB - O76031 (CLPX_HUMAN)

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Protein

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

Gene

CLPX

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

ATP-dependent specificity component of the Clp protease complex. Hydrolyzes ATP. Targets specific substrates for degradation by the Clp complex (PubMed:11923310, PubMed:22710082). Can perform chaperone functions in the absence of CLPP. Enhances the DNA-binding activity of TFAM and is required for maintaining a normal mitochondrial nucleoid structure (PubMed:22841477). ATP-dependent unfoldase that stimulates the incorporation of the pyridoxal phosphate cofactor into 5-aminolevulinate synthase, thereby activating 5-aminolevulinate (ALA) synthesis, the first step in heme biosynthesis. Important for efficient erythropoiesis through upregulation of heme biosynthesis (PubMed:25957689).4 Publications

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri105 – 130C4-typeAdd BLAST26
Nucleotide bindingi294 – 301ATPBy similarity8

GO - Molecular functioni

  • ATPase activity Source: UniProtKB
  • ATP binding Source: UniProtKB
  • metal ion binding Source: UniProtKB-KW
  • peptidase activator activity Source: UniProtKB
  • unfolded protein binding Source: InterPro
Complete GO annotation...

GO - Biological processi

  • protein folding Source: InterPro
  • proteolysis involved in cellular protein catabolic process Source: UniProtKB
Complete GO annotation...

Keywordsi

Molecular functionChaperone
LigandATP-binding, Metal-binding, Nucleotide-binding, Zinc

Names & Taxonomyi

Protein namesi
Recommended name:
ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial
Gene namesi
Name:CLPX
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
Proteomesi
  • UP000005640 Componenti: Chromosome 15

Organism-specific databases

HGNCiHGNC:2088. CLPX.

Subcellular locationi

GO - Cellular componenti

  • cytosol Source: HPA
  • endopeptidase Clp complex Source: UniProtKB
  • mitochondrial endopeptidase Clp complex Source: UniProtKB
  • mitochondrial inner membrane Source: UniProtKB
  • mitochondrial matrix Source: UniProtKB
  • mitochondrial nucleoid Source: BHF-UCL
  • mitochondrion Source: UniProtKB
  • nucleoplasm Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion, Mitochondrion nucleoid

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi359E → A: Abolishes ATP hydrolysis. 1 Publication1

Organism-specific databases

OpenTargetsiENSG00000166855.
PharmGKBiPA26614.

Polymorphism and mutation databases

BioMutaiCLPX.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 56MitochondrionSequence analysisAdd BLAST56
ChainiPRO_000000551857 – 633ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrialAdd BLAST577

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei437N6-acetyllysineCombined sources1
Modified residuei617PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiO76031.
MaxQBiO76031.
PaxDbiO76031.
PeptideAtlasiO76031.
PRIDEiO76031.

PTM databases

iPTMnetiO76031.
PhosphoSitePlusiO76031.

Expressioni

Tissue specificityi

Higher expression in skeletal muscle and heart and to a lesser extent in liver, brain, placenta, lung, kidney and pancreas.1 Publication

Gene expression databases

BgeeiENSG00000166855.
CleanExiHS_CLPX.
ExpressionAtlasiO76031. baseline and differential.
GenevisibleiO76031. HS.

Organism-specific databases

HPAiHPA040262.
HPA048199.

Interactioni

Subunit structurei

Homohexamer that forms a ring structure; this hexamerization requires ATP binding. Component of the Clp complex formed by the assembly of two CLPP heptameric rings with two CLPX hexameric rings, giving rise to a symmetrical structure with two central CLPP rings flanked by a CLPX ring at either end of the complex. Interacts with TFAM.5 Publications

GO - Molecular functioni

Complete GO annotation...

Protein-protein interaction databases

BioGridi116056. 51 interactors.
DIPiDIP-50293N.
IntActiO76031. 19 interactors.
MINTiMINT-3002168.
STRINGi9606.ENSP00000300107.

Structurei

3D structure databases

ProteinModelPortaliO76031.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the ClpX chaperone family.Curated

Zinc finger

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri105 – 130C4-typeAdd BLAST26

Keywords - Domaini

Transit peptide, Zinc-finger

Phylogenomic databases

eggNOGiKOG0745. Eukaryota.
COG1219. LUCA.
GeneTreeiENSGT00390000017625.
HOGENOMiHOG000010093.
HOVERGENiHBG004940.
InParanoidiO76031.
KOiK03544.
OMAiSPMDNDQ.
OrthoDBiEOG091G07XR.
PhylomeDBiO76031.
TreeFamiTF312884.

Family and domain databases

InterProiView protein in InterPro
IPR003593. AAA+_ATPase.
IPR003959. ATPase_AAA_core.
IPR019489. Clp_ATPase_C.
IPR004487. Clp_protease_ATP-bd_su_ClpX.
IPR027417. P-loop_NTPase.
PANTHERiPTHR11262. PTHR11262. 1 hit.
PfamiView protein in Pfam
PF07724. AAA_2. 1 hit.
PF10431. ClpB_D2-small. 1 hit.
SMARTiView protein in SMART
SM00382. AAA. 1 hit.
SM01086. ClpB_D2-small. 1 hit.
SUPFAMiSSF52540. SSF52540. 2 hits.
TIGRFAMsiTIGR00382. clpX. 1 hit.

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

O76031-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MPSCGACTCG AAAVRLITSS LASAQRGISG GRIHMSVLGR LGTFETQILQ 
        60         70         80         90        100
RAPLRSFTET PAYFASKDGI SKDGSGDGNK KSASEGSSKK SGSGNSGKGG 
       110        120        130        140        150
NQLRCPKCGD LCTHVETFVS STRFVKCEKC HHFFVVLSEA DSKKSIIKEP 
       160        170        180        190        200
ESAAEAVKLA FQQKPPPPPK KIYNYLDKYV VGQSFAKKVL SVAVYNHYKR 
       210        220        230        240        250
IYNNIPANLR QQAEVEKQTS LTPRELEIRR REDEYRFTKL LQIAGISPHG 
       260        270        280        290        300
NALGASMQQQ VNQQIPQEKR GGEVLDSSHD DIKLEKSNIL LLGPTGSGKT 
       310        320        330        340        350
LLAQTLAKCL DVPFAICDCT TLTQAGYVGE DIESVIAKLL QDANYNVEKA 
       360        370        380        390        400
QQGIVFLDEV DKIGSVPGIH QLRDVGGEGV QQGLLKLLEG TIVNVPEKNS 
       410        420        430        440        450
RKLRGETVQV DTTNILFVAS GAFNGLDRII SRRKNEKYLG FGTPSNLGKG 
       460        470        480        490        500
RRAAAAADLA NRSGESNTHQ DIEEKDRLLR HVEARDLIEF GMIPEFVGRL 
       510        520        530        540        550
PVVVPLHSLD EKTLVQILTE PRNAVIPQYQ ALFSMDKCEL NVTEDALKAI 
       560        570        580        590        600
ARLALERKTG ARGLRSIMEK LLLEPMFEVP NSDIVCVEVD KEVVEGKKEP 
       610        620        630 
GYIRAPTKES SEEEYDSGVE EEGWPRQADA ANS
Length:633
Mass (Da):69,224
Last modified:July 11, 2001 - v2
Checksum:iCF46A6DC0DDBF022
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti21L → P in BAF85005 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_048826488I → T. Corresponds to variant dbSNP:rs35754835Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ006267 mRNA. Translation: CAA06933.2.
AJ276980
, AJ276981, AJ276966, AJ276967, AJ276968, AJ276969, AJ276970, AJ276971, AJ276972, AJ276973, AJ276974, AJ276975, AJ276976, AJ276977 Genomic DNA. Translation: CAC01291.1.
AK292316 mRNA. Translation: BAF85005.1.
CH471082 Genomic DNA. Translation: EAW77715.1.
BC130373 mRNA. Translation: AAI30374.1.
BC136487 mRNA. Translation: AAI36488.1.
CCDSiCCDS10202.1.
RefSeqiNP_006651.2. NM_006660.4.
UniGeneiHs.113823.

Genome annotation databases

EnsembliENST00000300107; ENSP00000300107; ENSG00000166855.
GeneIDi10845.
KEGGihsa:10845.
UCSCiuc002aom.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.

Entry informationi

Entry nameiCLPX_HUMAN
AccessioniPrimary (citable) accession number: O76031
Secondary accession number(s): A1L428
, A8K8F1, B9EGI8, Q9H4D9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 30, 2000
Last sequence update: July 11, 2001
Last modified: May 10, 2017
This is version 153 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 15
    Human chromosome 15: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families