CLPX

Protein identityUniProt · HPA

UniProt accession

O76031

Gene

CLPX

Ensembl

ENSG00000166855

Chromosome

15

Canonical length

633 aa

Protein class

Disease related genes, Human disease related genes, Predicted intracellular proteins

Subcellular location

Nucleoplasm,Mitochondria,Cytosol,Equatorial segment,Perinuclear theca

Quaternary structure

Homohexamer

OverviewNCBI Gene

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]

Canonical amino-acid sequenceUniProt

633 residues, UniProt reviewed canonical sequence.

>O76031|CLPX
     1  MPSCGACTCG AAAVRLITSS LASAQRGISG GRIHMSVLGR LGTFETQILQ RAPLRSFTET
    61  PAYFASKDGI SKDGSGDGNK KSASEGSSKK SGSGNSGKGG NQLRCPKCGD LCTHVETFVS
   121  STRFVKCEKC HHFFVVLSEA DSKKSIIKEP ESAAEAVKLA FQQKPPPPPK KIYNYLDKYV
   181  VGQSFAKKVL SVAVYNHYKR IYNNIPANLR QQAEVEKQTS LTPRELEIRR REDEYRFTKL
   241  LQIAGISPHG NALGASMQQQ VNQQIPQEKR GGEVLDSSHD DIKLEKSNIL LLGPTGSGKT
   301  LLAQTLAKCL DVPFAICDCT TLTQAGYVGE DIESVIAKLL QDANYNVEKA QQGIVFLDEV
   361  DKIGSVPGIH QLRDVGGEGV QQGLLKLLEG TIVNVPEKNS RKLRGETVQV DTTNILFVAS
   421  GAFNGLDRII SRRKNEKYLG FGTPSNLGKG RRAAAAADLA NRSGESNTHQ DIEEKDRLLR
   481  HVEARDLIEF GMIPEFVGRL PVVVPLHSLD EKTLVQILTE PRNAVIPQYQ ALFSMDKCEL
   541  NVTEDALKAI ARLALERKTG ARGLRSIMEK LLLEPMFEVP NSDIVCVEVD KEVVEGKKEP
   601  GYIRAPTKES SEEEYDSGVE EEGWPRQADA ANS

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against CLPX can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.45

Highest tissue expression

31 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about CLPX.

Disease | AllUniProt

Conditions CLPX is implicated in, by any mechanism.

• Protoporphyria, erythropoietic, 2 (EPP2) MIM:618015

Disease | GeneticClinVar

1 pathogenic / likely-pathogenic of 166 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Protoporphyria, erythropoietic, 2

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.34

gnomAD pLI

0.92

gnomAD missense Z

1.81

DepMap mean gene effect

-0.24

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 4% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• ATP metabolic process
• proteolysis
• proteolysis involved in protein catabolic process

Molecular functions

• ATP binding
• ATP hydrolysis activity
• ATP-dependent protein folding chaperone
• peptidase activator activity
• protein dimerization activity
• unfolded protein binding
• zinc ion binding

Cellular components

• cytosol
• endopeptidase Clp complex
• mitochondrial inner membrane
• mitochondrial matrix
• mitochondrial nucleoid
• mitochondrion
• nucleoplasm
• perinuclear theca
• mitochondrial endopeptidase Clp complex

Protein domainsUniProt · Pfam · InterPro

• AAA+ ATPase domain
• ATPase, AAA-type, core
• Clp ATPase, C-terminal
• P-loop containing nucleoside triphosphate hydrolase
• AAA domain (Cdc48 subfamily)
• C-terminal, D2-small domain, of ClpB protein
• Clp protease, ATP-binding subunit ClpX
• ATP-dependent Clp protease ATP-binding subunit ClpX
• ATP-dependent ClpX-like chaperone, zinc ribbon domain
• ClpX-like, zinc ribbon domain
• CLPX-like, zinc ribbon domain

KeywordsUniProt

• Acetylation
• ATP-binding
• Chaperone
• Hydrolase
• Metal-binding
• Mitochondrion
• Mitochondrion nucleoid
• Nucleotide-binding
• Phosphoprotein
• Transit peptide
• Zinc

InteractionsUniProt · HPA

Protein binding partners of CLPX in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• TFAM