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FLASH GENE
Symbol HSCB contributors: mct - updated : 08-04-2016
HGNC name HscB iron-sulfur cluster co-chaperone homolog (E. coli)
HGNC id 28913
Location 22q12.1      Physical location : 29.138.042 - 29.153.488
Synonym name
  • DnaJ (Hsp40) homolog, subfamily C, member 20
  • HscB iron-sulfur cluster co-chaperone homolog (E. coli)
  • iron-sulfur cluster co-chaperone protein HscB, mitochondrial
  • J-type co-chaperone HSC20
  • Synonym symbol(s) HSC20, DNAJC20, Jac1, MGC2637, MGC74462, dJ366L4.2
    DNA
    TYPE functioning gene
    STRUCTURE 15.45 kb     6 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    6 - 1106 27.4 235 - 2008 18713742
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Nervousbrain   lowly Homo sapiens
    Respiratorylung   highly Homo sapiens
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • cysteine-rich N-terminal domain, important for the integrity and function of the human co-chaperone , and a DnaJ domain
  • a putative mitochondrial leader sequence (AAs 1–35) in the N-terminus of the human protein with a potential cleavage site between AAs 21 and 22
  • a functional J-domain that appears to be critical for the biological activity of HSCB
  • conserved patch in the C-domain is the principal binding site for ISCU and an HSCB_C domain at the C terminus (
  • HOMOLOGY
    interspecies ortholog to yeast Jac1p
    Homologene
    FAMILY
    CATEGORY chaperone/stress
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,cytoplasm,cytosolic
    text
  • endogenous HSCB was mainly mitochondrial
  • predominantly localizes on mitochondria, and this location is important for the function of HSCB in ISC biogenesis
  • basic FUNCTION
  • integral function in iron–sulfur cluster (ISC) biogenesis, which has an impact on both mitochondrial and cytosolic ISC-containing proteins
  • is a frataxin interactor that participates in mammalian iron–sulfur biogenesis
  • could stabilize frataxin in the mitochondria
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • integral component of the iron–sulfur cluster (ISC) biosynthetic machinery that is particularly important in the assembly of ISCs under conditions of oxidative stress
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interaction between ISCU and HSCB is critical for successful assembly of iron-sulfur clusters
  • interaction between IscU and HscB is critical for successful assembly of iron-sulfur clustersbinding partner ISCU and HSPA9
  • interacts with frataxin structurally and functionally and is important for iron–sulfur cluster (ISC) biogenesis and iron homeostasis
  • interacts with multiple proteins involved in ISC biogenesis including the ISCU ISC biogenesis complex and the HSPA9 ISC chaperone
  • activated the ATPase activity of HSPA9, and this activation was greatly increased by the addition of ISCU
  • preferentially binds and stabilizes the S-state of ISCU
  • interactions between HSCB and LYR motif-containing proteins (LYRM7) that are key to the assembly and function of complexes I, II, and III
  • HSCB, essential for Fe-S cluster biogenesis of SDHB, directly binds LYRM7, formerly described as a chaperone that stabilizes UQCRFS1 prior to its insertion into CIII
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --over  
    substantially protected cells from oxidative insults
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS