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FLASH GENE
Symbol CLPP contributors: mct - updated : 30-04-2015
HGNC name ClpP caseinolytic protease, ATP-dependent, proteolytic subunit homolog (E. coli)
HGNC id 2084
Corresponding disease
PRLTS3 Perrault Syndrome 3
Location 19p13.3      Physical location : 6.361.462 - 6.368.915
Synonym name
  • caseinolytic protease ATP dependent
  • ATP-dependent protease ClpAP, proteolytic subunit
  • Synonym symbol(s) ClpAP
    EC.number 3.4.21.92
    DNA
    TYPE functioning gene
    STRUCTURE 7.45 kb     6 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked   status confirmed
    Physical map
    MGC39581 19p13.3 hypothetical protein MGC39581 MGC24975 19p13.3 hypothetical protein MGC24975 LOC56931 19p13.3 hypothetical protein from EUROIMAGE 1967720 FUT6 19p13.3 fucosyltransferase 6 (alpha (1,3) fucosyltransferase) NRTN 19p13.3 neurturin FUT3 19p13.3 fucosyltransferase 3 (galactoside 3(4)-L-fucosyltransferase, Lewis blood group included) FUT5 19p13.3 fucosyltransferase 5 (alpha (1,3) fucosyltransferase) NDUFA11 19p13.3 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 11, 14.7kDa CAPS 19p13.3 calcyphosine RANBP3 19p13.3 RAN binding protein 3 RFX2 19p13.3 regulatory factor X, 2 (influences HLA class II expression) BGR 19p13.3 bubblegum related protein LOC388497 19 LOC388497 MLLT1 19p13.3 myeloid/lymphoid or mixed-lineage leukemia (trithorax homolog, Drosophila); translocated to, 1 ASAH3 19p13.3 N-acylsphingosine amidohydrolase (alkaline ceramidase) 3 CLPP 19p13.2 ClpP caseinolytic protease, ATP-dependent, proteolytic subunit homolog (E. coli) SPATA11 19p13.3 spermatogenesis associated 11 PSPN 19p13.3 persephin GTF2F1 19p13.3 general transcription factor IIF, polypeptide 1, 74kDa LOC390877 19 similar to adenylate kinase (EC 2.7.4.3), cytosolic - common carp KHSRP 19p13.3 KH-type splicing regulatory protein (FUSE binding protein 2) MGC34725 19p13.3 hypothetical protein MGC34725 MGC2615 19p13.3 hypothetical protein MGC2615 CRB3 19p13.3 crumbs homolog 3 (Drosophila) FLJ22757 TUBB5 19p13.3 tubulin, beta, 5 TNFSF9 19p13.3 tumor necrosis factor (ligand) superfamily, member 9 TNFSF7 19p13 tumor necrosis factor (ligand) superfamily, member 7 LOC390878 19 similar to 60S ribosomal protein L7 TNFSF14 19p13.3 tumor necrosis factor (ligand) superfamily, member 14 C3 19p13.3 complement component 3 GPR108 19p13.3 G protein-coupled receptor 108 TRIP10 19p13.3 thyroid hormone receptor interactor 10 SH2D3A 19p13.3 SH2 domain containing 3A VAV1 19p13.3 vav 1 oncogene EMR1 19p13.3 egf-like module containing, mucin-like, hormone receptor-like sequence 1 EMR4 19p13.3 egf-like module containing, mucin-like, hormone receptor-like 4
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    6 - 1194 - 277 - 2005 16115876
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart    
    Digestiveliver    
    Endocrinepancreas    
    Lymphoid/Immunelymph node   highly
    Reproductivefemale systemovary  highly
    Respiratorylung   highly
    Skin/Tegumentskin   highly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Muscularstriatumcardiac  
    Muscularstriatumskeletal  
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • N-terminus acts as a "gate" controlling substrate access to the active sites
  • mono polymer heptamer
    isoforms Precursor
    HOMOLOGY
    interspecies homolog to E.coli Clpp
    homolog to murine Clpp
    homolog to C.elegans ZK970.2
    Homologene
    FAMILY peptidase family S14 or CLPP family
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    basic FUNCTION
  • caseinolytic, ATP dependent protease involved in degradation of incorrectly folded or unfolded proteins
  • hydrolyzing proteins into small peptides in the presence of ATP and magnesium
  • LONP1 and CLPP have been shown to degrade unfolded and damaged proteins in the matrix of mitochondria
  • has an essential role in determining the rate of mitochondrial protein synthesis by regulating the level of mitoribosome assembly
  • CELLULAR PROCESS protein, degradation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • proteolytic subunit of CLP
  • polypeptide, composed of two heptameric rings with 7 fold symmetry
  • INTERACTION
    DNA
    RNA
    small molecule metal binding, nucleotide,
  • magnesium Mg2+
  • ATP
  • protein
  • correlative effect of LONP1 and CLPP upregulation on loss of mitochondrial Fe-S proteins during the progression of the disease may suggest that Fe-S proteins are potential targets of LONP1 and CLPP proteases in FRDA
  • cell & other associated with the inner mitochondrial membrane
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) PRLTS3
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS