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Aliases for CLPP Gene

Aliases for CLPP Gene

  • Caseinolytic Mitochondrial Matrix Peptidase Proteolytic Subunit 2 3
  • Endopeptidase Clp 3 4
  • EC 3.4.21.92 4 64
  • PRLTS3 3 6
  • ClpP (Caseinolytic Protease, ATP-Dependent, Proteolytic Subunit, E. Coli) Homolog 2
  • ClpP Caseinolytic Peptidase, ATP-Dependent, Proteolytic Subunit Homolog (E. Coli) 2
  • ClpP Caseinolytic Protease, ATP-Dependent, Proteolytic Subunit Homolog (E. Coli) 2
  • ClpP Caseinolytic Peptidase, ATP-Dependent, Proteolytic Subunit Homolog 3
  • ClpP Caseinolytic Protease, ATP-Dependent, Proteolytic Subunit Homolog 3
  • Putative ATP-Dependent Clp Protease Proteolytic Subunit, Mitochondrial 3
  • ClpP Caseinolytic Peptidase ATP-Dependent, Proteolytic Subunit 3
  • ATP-Dependent Clp Protease Proteolytic Subunit, Mitochondrial 3
  • ATP-Dependent Protease ClpAP, Proteolytic Subunit, Human 3
  • ATP-Dependent Protease ClpAP (E. Coli) 2
  • Proteolytic Subunit 2
  • DFNB81 3
  • Human 2

External Ids for CLPP Gene

Summaries for CLPP Gene

Entrez Gene Summary for CLPP Gene

  • The protein encoded by this gene belongs to the peptidase family S14 and hydrolyzes proteins into small peptides in the presence of ATP and magnesium. The protein is transported into mitochondrial matrix and is associated with the inner mitochondrial membrane. [provided by RefSeq, Jul 2008]

GeneCards Summary for CLPP Gene

CLPP (Caseinolytic Mitochondrial Matrix Peptidase Proteolytic Subunit) is a Protein Coding gene. Diseases associated with CLPP include perrault syndrome 3 and perrault syndrome. GO annotations related to this gene include serine-type endopeptidase activity and peptidase activity.

UniProtKB/Swiss-Prot for CLPP Gene

  • 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.

Gene Wiki entry for CLPP Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for CLPP Gene

Genomics for CLPP Gene

Genomic Location for CLPP Gene

Start:
6,361,452 bp from pter
End:
6,368,908 bp from pter
Size:
7,457 bases
Orientation:
Plus strand

Genomic View for CLPP Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for CLPP Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for CLPP Gene

Regulatory Elements for CLPP Gene

Proteins for CLPP Gene

  • Protein details for CLPP Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Q16740-CLPP_HUMAN
    Recommended name:
    ATP-dependent Clp protease proteolytic subunit, mitochondrial
    Protein Accession:
    Q16740
    Secondary Accessions:
    • B2R4W5

    Protein attributes for CLPP Gene

    Size:
    277 amino acids
    Molecular mass:
    30180 Da
    Quaternary structure:
    • Fourteen CLPP subunits assemble into 2 heptameric rings which stack back to back to give a disk-like structure with a central cavity. 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.

    Three dimensional structures from OCA and Proteopedia for CLPP Gene

neXtProt entry for CLPP Gene

Proteomics data for CLPP Gene at MOPED

Post-translational modifications for CLPP Gene

  • Modification sites at PhosphoSitePlus
  • Ubiquitination at Lys203

Other Protein References for CLPP Gene

Domains for CLPP Gene

Gene Families for CLPP Gene

HGNC:
  • AATP :ATPases / AAA-type

Protein Domains for CLPP Gene

UniProtKB/Swiss-Prot:

CLPP_HUMAN
Family:
  • Belongs to the peptidase S14 family.:
    • Q16740
genes like me logo Genes that share domains with CLPP: view

Function for CLPP Gene

Molecular function for CLPP Gene

GENATLAS Biochemistry: ATP dependent protease involved in degradation of incorreclly folded or unfolded proteins,catalytic subunit,ClpP E coli homolog,highly expressed in skeletal muscle,heart,liver,pancreas,located in the mitochondrial matrix
UniProtKB/Swiss-Prot CatalyticActivity: Hydrolysis of proteins to small peptides in the presence of ATP and magnesium. Alpha-casein is the usual test substrate. In the absence of ATP, only oligopeptides shorter than five residues are hydrolyzed (such as succinyl-Leu-Tyr- -NHMec; and Leu-Tyr-Leu- -Tyr-Trp, in which cleavage of the -Tyr- -Leu- and -Tyr- -Trp bonds also occurs).
UniProtKB/Swiss-Prot 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.

Enzyme Numbers (IUBMB) for CLPP Gene

Gene Ontology (GO) - Molecular Function for CLPP Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004252 serine-type endopeptidase activity IDA 11923310
GO:0005515 protein binding IPI 11923310
GO:0008233 peptidase activity TAS 8543061
GO:0042802 identical protein binding IEA --
genes like me logo Genes that share ontologies with CLPP: view
genes like me logo Genes that share phenotypes with CLPP: view

miRNA for CLPP Gene

miRTarBase miRNAs that target CLPP

No data available for Animal Models , Transcription Factor Targeting and HOMER Transcription for CLPP Gene

Localization for CLPP Gene

Subcellular locations from UniProtKB/Swiss-Prot for CLPP Gene

Mitochondrion matrix.

Subcellular locations from

COMPARTMENTS
Jensen Localization Image for CLPP Gene COMPARTMENTS Subcellular localization image for CLPP gene
Compartment Confidence
mitochondrion 5
chloroplast 3
nucleus 1

Gene Ontology (GO) - Cellular Components for CLPP Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IDA --
GO:0005759 mitochondrial matrix IDA 10525407
GO:0009368 endopeptidase Clp complex IDA 11923310
genes like me logo Genes that share ontologies with CLPP: view

Pathways for CLPP Gene

SuperPathways for CLPP Gene

No Data Available

Gene Ontology (GO) - Biological Process for CLPP Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006508 proteolysis --
GO:0051260 protein homooligomerization IEA --
GO:0051603 proteolysis involved in cellular protein catabolic process IDA 11923310
genes like me logo Genes that share ontologies with CLPP: view

No data available for Pathways by source for CLPP Gene

Compounds for CLPP Gene

(1) HMDB Compounds for CLPP Gene

Compound Synonyms Cas Number PubMed IDs
Magnesium
  • Magnesium
7439-95-4

(1) Drugbank Compounds for CLPP Gene

Compound Synonyms Cas Number Type Actions PubMed IDs
N-Formylmethionine
target

(1) Novoseek inferred chemical compound relationships for CLPP Gene

Compound -log(P) Hits PubMed IDs
serine 37.5 2
genes like me logo Genes that share compounds with CLPP: view

Transcripts for CLPP Gene

mRNA/cDNA for CLPP Gene

Unigene Clusters for CLPP Gene

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

Alternative Splicing Database (ASD) splice patterns (SP) for CLPP Gene

ExUns: 1 ^ 2a · 2b · 2c ^ 3a · 3b · 3c ^ 4 ^ 5 ^ 6a · 6b
SP1: - - -
SP2: -
SP3: - -
SP4:
SP5: -

Relevant External Links for CLPP Gene

GeneLoc Exon Structure for
CLPP
ECgene alternative splicing isoforms for
CLPP

Expression for CLPP Gene

mRNA expression in normal human tissues for CLPP Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for CLPP Gene

SOURCE GeneReport for Unigene cluster for CLPP Gene Hs.515092

mRNA Expression by UniProt/SwissProt for CLPP Gene

Q16740-CLPP_HUMAN
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.
genes like me logo Genes that share expressions with CLPP: view

Orthologs for CLPP Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for CLPP Gene

Organism Taxonomy Gene Similarity Type Details
chimpanzee
(Pan troglodytes)
Mammalia CLPP 36
  • 98.92 (n)
  • 98.92 (a)
CLPP 37
  • 99 (a)
OneToOne
cow
(Bos Taurus)
Mammalia CLPP 36
  • 89.67 (n)
  • 91.51 (a)
CLPP 37
  • 91 (a)
OneToOne
dog
(Canis familiaris)
Mammalia CLPP 36
  • 90.2 (n)
  • 91.54 (a)
CLPP 37
  • 82 (a)
OneToOne
mouse
(Mus musculus)
Mammalia Clpp 36
  • 83.66 (n)
  • 90.98 (a)
Clpp 16
Clpp 37
  • 87 (a)
OneToOne
platypus
(Ornithorhynchus anatinus)
Mammalia CLPP 37
  • 92 (a)
OneToOne
rat
(Rattus norvegicus)
Mammalia Clpp 36
  • 81.45 (n)
  • 87.16 (a)
lizard
(Anolis carolinensis)
Reptilia CLPP 37
  • 77 (a)
OneToOne
African clawed frog
(Xenopus laevis)
Amphibia Xl.11723 36
tropical clawed frog
(Silurana tropicalis)
Amphibia clpp 36
  • 73.48 (n)
  • 86.36 (a)
Str.5533 36
zebrafish
(Danio rerio)
Actinopterygii clpp 36
  • 72.83 (n)
  • 82.82 (a)
clpp 37
  • 73 (a)
OneToOne
wufj37a05 36
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP009019 36
  • 67.61 (n)
  • 74.53 (a)
fruit fly
(Drosophila melanogaster)
Insecta CG5045 36
  • 68.16 (n)
  • 70.65 (a)
CG5045 37
  • 58 (a)
OneToOne
CG5045 38
  • 70 (a)
worm
(Caenorhabditis elegans)
Secernentea clpp-1 36
  • 55.19 (n)
  • 59.91 (a)
clpp-1 37
  • 57 (a)
OneToOne
ZK970.2 38
  • 65 (a)
thale cress
(Arabidopsis thaliana)
eudicotyledons NCLPP7 36
  • 58 (n)
  • 60 (a)
corn
(Zea mays)
Liliopsida Zm.16493 36
rice
(Oryza sativa)
Liliopsida Os.4948 36
Os02g0634500 36
  • 68.01 (n)
  • 65.33 (a)
wheat
(Triticum aestivum)
Liliopsida Ta.9277 36
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.4342 36
sea squirt
(Ciona savignyi)
Ascidiacea CSA.3464 37
  • 61 (a)
OneToOne
Species with no ortholog for CLPP:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • baker's yeast (Saccharomyces cerevisiae)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • chicken (Gallus gallus)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • oppossum (Monodelphis domestica)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)

Evolution for CLPP Gene

ENSEMBL:
Gene Tree for CLPP (if available)
TreeFam:
Gene Tree for CLPP (if available)

Paralogs for CLPP Gene

No data available for Paralogs for CLPP Gene

Variants for CLPP Gene

Sequence variations from dbSNP and Humsavar for CLPP Gene

SNP ID Clin Chr 19 pos Sequence Context AA Info Type MAF
rs1049343 -- 6,368,863(+) TCCAG(C/G)TGAGA utr-variant-3-prime
rs2304197 -- 6,366,215(+) GACGC(C/T)GATAC intron-variant
rs2860168 -- 6,366,138(-) GGCTG(A/G)TGGGC intron-variant
rs7245514 -- 6,363,482(+) ACATC(C/T)GGCAA intron-variant
rs7246979 -- 6,368,264(+) cggcc(A/G)ccttc intron-variant

Structural Variations from Database of Genomic Variants (DGV) for CLPP Gene

Variant ID Type Subtype PubMed ID
nsv910911 CNV Loss 21882294
dgv3698n71 CNV Loss 21882294
nsv910916 CNV Loss 21882294

Relevant External Links for CLPP Gene

HapMap Linkage Disequilibrium report
CLPP
Human Gene Mutation Database (HGMD)
CLPP

Disorders for CLPP Gene

(1) OMIM Diseases for CLPP Gene (601119)

UniProtKB/Swiss-Prot

CLPP_HUMAN
  • 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:0000269 PubMed:23541340}. Note=The disease is caused by mutations affecting the gene represented in this entry.

(1) University of Copenhagen DISEASES for CLPP Gene

(1) Novoseek inferred disease relationships for CLPP Gene

Disease -log(P) Hits PubMed IDs
shock 33.8 4

Relevant External Links for CLPP

Genetic Association Database (GAD)
CLPP
Human Genome Epidemiology (HuGE) Navigator
CLPP
genes like me logo Genes that share disorders with CLPP: view

Publications for CLPP Gene

  1. Human ClpP protease: cDNA sequence, tissue-specific expression and chromosomal assignment of the gene. (PMID: 8543061) Bross P. … Gregersen N. (FEBS Lett. 1995) 2 3 4 23
  2. Decreased expression of the mitochondrial matrix proteases Lon and ClpP in cells from a patient with hereditary spastic paraplegia (SPG13). (PMID: 18378094) Hansen J. … Bross P. (Neuroscience 2008) 3 23
  3. The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). (PMID: 15489334) Gerhard D.S. … Malek J. (Genome Res. 2004) 3 4
  4. Crystallography and mutagenesis point to an essential role for the N- terminus of human mitochondrial ClpP. (PMID: 15522782) Kang S.G. … Ahvazi B. (J. Struct. Biol. 2004) 3 4
  5. Human mitochondrial ClpP is a stable heptamer that assembles into a tetradecamer in the presence of ClpX. (PMID: 16115876) Kang S.G. … Maurizi M.R. (J. Biol. Chem. 2005) 3 4

Products for CLPP Gene

Sources for CLPP Gene

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