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Aliases for CLPP Gene

Aliases for CLPP Gene

  • Caseinolytic Mitochondrial Matrix Peptidase Proteolytic Subunit 2 3 5
  • Endopeptidase Clp 3 4
  • EC 3.4.21.92 4 63
  • 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 Protease ClpAP, Proteolytic Subunit, Human 3
  • ATP-Dependent Protease ClpAP (E. Coli) 2
  • Proteolytic Subunit 2
  • DFNB81 3
  • PRLTS3 3
  • Human 2

External Ids for CLPP Gene

Previous GeneCards Identifiers for CLPP Gene

  • GC19P006455
  • GC19P006301
  • GC19P006313
  • GC19P006314
  • GC19P006315
  • GC19P006121
  • GC19P006367
  • GC19P006363
  • GC19P006364

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. Among its related pathways are Glucose / Energy Metabolism and Neuroscience. GO annotations related to this gene include identical protein binding 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

Regulatory Elements for CLPP Gene

Enhancers for CLPP Gene
GeneHancer Identifier Score Enhancer Sources TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor Binding Sites within enhancer Other Gene Targets for Enhancer

Enhancers around CLPP on UCSC Golden Path with GeneCards custom track

Promoters for CLPP Gene
Ensembl Regulatory Elements (ENSRs) TSS Distance (bp) Size (bp) Binding Sites for Transcription Factors within promoters

ENSRs around CLPP on UCSC Golden Path with GeneCards custom track

Genomic Location for CLPP Gene

Chromosome:
19
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

Genes around CLPP on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
CLPP Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for CLPP Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence 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

  • Ubiquitination at Lys 203
  • Modification sites at PhosphoSitePlus

Other Protein References for CLPP Gene

Antibody Products

  • Cell Signaling Technology (CST) Antibodies for CLPP (CLPP)
  • Abcam antibodies for CLPP

Domains & Families for CLPP Gene

Gene Families for CLPP Gene

Protein Domains for CLPP Gene

Suggested Antigen Peptide Sequences for CLPP Gene

GenScript: Design optimal peptide antigens:

Graphical View of Domain Structure for InterPro Entry

Q16740

UniProtKB/Swiss-Prot:

CLPP_HUMAN :
  • Belongs to the peptidase S14 family.
Family:
  • Belongs to the peptidase S14 family.
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:0008233 peptidase activity TAS 8543061
genes like me logo Genes that share ontologies with CLPP: view
genes like me logo Genes that share phenotypes with CLPP: view

Human Phenotype Ontology for CLPP Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

Animal Models for CLPP Gene

MGI Knock Outs for CLPP:
  • Clpp tm1a(EUCOMM)Wtsi

Animal Model Products

  • Taconic Biosciences Mouse Models for CLPP

No data available for Transcription Factor Targets 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:0009368 endopeptidase Clp complex IDA 11923310
genes like me logo Genes that share ontologies with CLPP: view

Pathways & Interactions for CLPP Gene

genes like me logo Genes that share pathways with CLPP: view

Pathways by source for CLPP Gene

2 Cell Signaling Technology pathways for CLPP Gene

Gene Ontology (GO) - Biological Process for CLPP Gene

None

No data available for SIGNOR curated interactions for CLPP Gene

Drugs & Compounds for CLPP Gene

(3) Drugs for CLPP Gene - From: DrugBank, HMDB, and Novoseek

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
N-Formylmethionine Experimental Pharma Target 0
Magnesium Nutra 0
genes like me logo Genes that share compounds with CLPP: view

Transcripts 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

Protein differential expression in normal tissues from HIPED for CLPP Gene

This gene is overexpressed in Nasal epithelium (11.0) and Peripheral blood mononuclear cells (7.4).

Integrated Proteomics: protein expression in normal tissues and cell lines 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 expression patterns with CLPP: view

Protein tissue co-expression partners for CLPP Gene

- Elite partner

Primer Products

No data available for mRNA differential expression in normal tissues for CLPP Gene

Orthologs for CLPP Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for CLPP Gene

Organism Taxonomy Gene Similarity Type Details
cow
(Bos Taurus)
Mammalia CLPP 35
  • 89.67 (n)
  • 91.51 (a)
CLPP 36
  • 91 (a)
OneToOne
dog
(Canis familiaris)
Mammalia CLPP 35
  • 90.2 (n)
  • 91.54 (a)
CLPP 36
  • 82 (a)
OneToOne
mouse
(Mus musculus)
Mammalia Clpp 35
  • 83.66 (n)
  • 90.98 (a)
Clpp 16
Clpp 36
  • 87 (a)
OneToOne
chimpanzee
(Pan troglodytes)
Mammalia CLPP 35
  • 98.92 (n)
  • 98.92 (a)
CLPP 36
  • 99 (a)
OneToOne
rat
(Rattus norvegicus)
Mammalia Clpp 35
  • 81.45 (n)
  • 87.16 (a)
platypus
(Ornithorhynchus anatinus)
Mammalia CLPP 36
  • 92 (a)
OneToOne
lizard
(Anolis carolinensis)
Reptilia CLPP 36
  • 77 (a)
OneToOne
tropical clawed frog
(Silurana tropicalis)
Amphibia clpp 35
  • 73.48 (n)
  • 86.36 (a)
Str.5533 35
African clawed frog
(Xenopus laevis)
Amphibia Xl.11723 35
zebrafish
(Danio rerio)
Actinopterygii clpp 35
  • 72.83 (n)
  • 82.82 (a)
wufj37a05 35
clpp 36
  • 73 (a)
OneToOne
fruit fly
(Drosophila melanogaster)
Insecta CG5045 37
  • 70 (a)
CG5045 35
  • 68.16 (n)
  • 70.65 (a)
CG5045 36
  • 58 (a)
OneToOne
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP009019 35
  • 67.61 (n)
  • 74.53 (a)
worm
(Caenorhabditis elegans)
Secernentea ZK970.2 37
  • 65 (a)
clpp-1 35
  • 55.19 (n)
  • 59.91 (a)
clpp-1 36
  • 57 (a)
OneToOne
thale cress
(Arabidopsis thaliana)
eudicotyledons NCLPP7 35
  • 58 (n)
  • 60 (a)
rice
(Oryza sativa)
Liliopsida Os.4948 35
Os02g0634500 35
  • 68.01 (n)
  • 65.33 (a)
wheat
(Triticum aestivum)
Liliopsida Ta.9277 35
corn
(Zea mays)
Liliopsida Zm.16493 35
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.4342 35
sea squirt
(Ciona savignyi)
Ascidiacea CSA.3464 36
  • 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
VAR_070092 Perrault syndrome 3 (PRLTS3)
VAR_070093 Perrault syndrome 3 (PRLTS3)
VAR_074160 Perrault syndrome 3 (PRLTS3)
rs7260547 -- 6,364,489(+) GACAC(A/G)ATGCA reference, synonymous-codon
rs8100489 -- 6,364,442(+) CTCCC(C/T)CGCCC 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

Variation tolerance for CLPP Gene

Residual Variation Intolerance Score: 29.1% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 0.14; 3.06% of all genes are more intolerant (likely to be disease-causing)

Relevant External Links for CLPP Gene

Human Gene Mutation Database (HGMD)
CLPP

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for CLPP Gene

Disorders for CLPP Gene

MalaCards: The human disease database

(4) MalaCards diseases for CLPP Gene - From: OMIM, ClinVar, GeneTests, Orphanet, Swiss-Prot, DISEASES, and GeneCards

Disorder Aliases PubMed IDs
perrault syndrome 3
  • prlts3
perrault syndrome
  • gonadal dysgenesis, xx type
legionaire disease
  • legionnaires' disease
learning disability
  • academic skill disorder
- elite association - COSMIC cancer census association via MalaCards
Search CLPP in MalaCards View complete list of genes associated with diseases

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, ECO:0000269 PubMed:25956234}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Relevant External Links for CLPP

Genetic Association Database (GAD)
CLPP
Human Genome Epidemiology (HuGE) Navigator
CLPP
Atlas of Genetics and Cytogenetics in Oncology and Haematology:
CLPP
genes like me logo Genes that share disorders with CLPP: view

No data available for Genatlas for CLPP Gene

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 67
  2. Human CLPP reverts the longevity phenotype of a fungal ClpP deletion strain. (PMID: 23360988) Fischer F. … Osiewacz H.D. (Nat Commun 2013) 2 3
  3. 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
  4. BioID-based Identification of Skp Cullin F-box (SCF)I^-TrCP1/2 E3 Ligase Substrates. (PMID: 25900982) Coyaud E. … Raught B. (Mol. Cell Proteomics 2015) 3
  5. Exome analysis identified a novel missense mutation in the CLPP gene in a consanguineous Saudi family expanding the clinical spectrum of Perrault Syndrome type-3. (PMID: 25956234) Ahmed S. … Al-Aama J.Y. (J. Neurol. Sci. 2015) 3

Products for CLPP Gene

Sources for CLPP Gene

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