InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: CLPP

Summary

InnateDB Protein

IDBP-21299.5

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

CLPP

Protein Name

ClpP caseinolytic peptidase, ATP-dependent, proteolytic subunit homolog (E. coli)

Synonyms

PRLTS3;

Species

Homo sapiens

Ensembl Protein

ENSP00000245816

InnateDB Gene

IDBG-21297 (CLPP)

Protein Structure

UniProt Annotation

Function

Protease component of the Clp complex that cleaves peptides and various proteins in an ATP-dependent process. Has low peptidase activity in the absence of CLPX. The Clp complex can degrade CSN1S1, CSN2 and CSN3, as well as synthetic peptides (in vitro) and may be responsible for a fairly general and central housekeeping function rather than for the degradation of specific substrates. {ECO:0000269PubMed:11923310, ECO:0000269PubMed:15522782}.

Subcellular Localization

Mitochondrion matrix {ECO:0000269PubMed:10525407}.

Disease Associations

Perrault syndrome 3 (PRLTS3) [MIM:614129]: A sex- influenced disorder characterized by sensorineural deafness in both males and females, and ovarian dysgenesis in females. Affected females have primary amenorrhea, streak gonads, and infertility, whereas affected males show normal pubertal development and are fertile. A spectrum of additional clinical features, including cerebellar ataxia, learning disability, and peripheral neuropathy, have been described in some PRLTS3 affected individuals. {ECO:0000269PubMed:23541340}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Detected in liver (at protein level). Predominantly expressed in skeletal muscle. Intermediate levels in heart, liver and pancreas. Low in brain, placenta, lung and kidney. {ECO:0000269PubMed:10525407, ECO:0000269PubMed:8543061}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 4 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.

Experimentally validated
Total	4 [view]
Protein-Protein	4 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	1 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004252	serine-type endopeptidase activity
GO:0005515	protein binding
GO:0008233	peptidase activity
GO:0042802	identical protein binding

Biological Process

GO:0006508	proteolysis
GO:0051260	protein homooligomerization
GO:0051603	proteolysis involved in cellular protein catabolic process

Cellular Component

GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix
GO:0009368	endopeptidase Clp complex

Protein Structure and Domains

PDB ID

InterPro

IPR001907 ATP-dependent Clp protease proteolytic subunit
IPR023562 Clp protease proteolytic subunit /Translocation-enhancing protein TepA
IPR029045 ClpP/crotonase-like domain

PFAM

PF00574

PRINTS

PR00127

PIRSF

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q16740