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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
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