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FLASH GENE
Symbol CLPB contributors: mct - updated : 25-02-2015
HGNC name ClpB caseinolytic peptidase B homolog (E. coli)
HGNC id 30664
Corresponding disease
MGCA7 3-methylglutaconic aciduria, type VII
Location 11q13.4      Physical location : 72.003.471 - 72.145.568
Synonym name
  • suppressor of potassium transport defect 3
  • caseinolytic peptidase B protein homolog
  • Synonym symbol(s) SKD3, HSP78, FLJ13152, HSP104
    DNA
    TYPE functioning gene
    STRUCTURE 142.26 kb     17 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    15 - 3095 - 648 - 2006 16834329
    16 - 3182 - 677 - 2006 16834329
    17 - 3272 - 707 - 2006 16834329
    18 - 3379 - 662 - 2006 16834329
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveintestinelarge intestinecolon  
     stomach    
    Endocrineadrenal gland    
    Nervousbrain   highly Homo sapiensAdult
    Reproductivefemale systembreastmammary gland  
     male systemtestis   
    Skin/Tegumentskin    
    Urinarykidney    
    Visualeye    
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Blood/Hematopoieticgranulocyte Homo sapiens
    cell lineage
    cell lines
    fluid/secretion blood, lymph
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • four ANK repeats in the N-terminal part of the protein might have evolved to ensure a more elaborate or more refined substrate recognition, to mediate the interaction with as yet unknown protein partners, or even to support a putative chaperone function
  • first 92 AAs are predicted to encode a mitochondrial targeting sequence
  • two AAA+ modules, that bind and hydrolyze ATP and construct the core of the hexameric ring
  • a specific C-terminal D2 domain, which is typical for AAA+ proteins involved in polypeptide chain threading through the hexamer central channel
  • mono polymer homomer , hexamer
    HOMOLOGY
    interspecies homolog to murine Clpb
    ortholog to rattus Skd3
    Homologene
    FAMILY
  • clpA/clpB family
  • AAA+ protein family
  • CATEGORY chaperone/stress , enzyme , regulatory
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    basic FUNCTION
  • may function as a regulatory ATPase and be related to secretion/protein trafficking process
  • protect cells from the devastating effects of protein inactivation and aggregation that arise after extreme heat stress
  • can passively thread soluble denatured proteins
  • CLPB hexamers remain associated during several ATP hydrolysis events required to partially or completely translocate substrates through the protein central channel, and CLPB dynamics is tuned by DnaK and substrate proteins
  • its function might be related to apoptosis
  • key role of CLPB in regulating protein folding inside the mitochondrial matrix
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS exocytosis transport , cellular trafficking transport
    PATHWAY
    metabolism
    signaling
    a component
  • forms a hexameric, ring-shaped complex that functions as a tightly regulated, ATP-powered molecular disaggregation machine
  • INTERACTION
    DNA
    RNA
    small molecule nucleotide,
    ATP binding
    protein
  • interacts biochemically with ATP2A2, known to be involved in apoptotic processes in severe congenital neutropenia
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MGCA7
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • suppression of clpb in zebrafish embryos induced a central nervous system phenotype that was consistent with cerebellar and cerebral atrophy