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FLASH GENE
Symbol BOLA3 contributors: mct/npt - updated : 27-06-2023
HGNC name bolA-like 3 (E. coli)
HGNC id 24415
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a mitochondrial targeting sequence
  • HOMOLOGY
    Homologene
    FAMILY
  • BolA-like family of proteins
  • CATEGORY regulatory
    SUBCELLULAR LOCALIZATION extracellular
        intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    intracellular,cytoplasm,cytosolic
    intracellular,nucleus,nucleoplasm,nuclear bodies
    basic FUNCTION
  • involved in cell proliferation or cell-cycle regulation
  • play essential roles in the production of [Fe-S] centers for the normal maturation of lipoate-containing 2-oxoacid dehydrogenases, and for the assembly of the respiratory chain complexes
  • NFU1- (and BOLA3-) dependent assembled clusters are directed to complex I and lipoate synthesis
  • with GLRX, help regulate iron metabolism
  • BOLA1 and BOLA3 are specific mitochondrial ISC assembly factors that facilitate [4Fe-4S] cluster insertion into a subset of mitochondrial proteins such as lipoate synthase and succinate dehydrogenase
  • acts as a crucial lynchpin connecting Fe-S-dependent oxidative respiration and glycine homeostasis with endothelial metabolic reprogramming critical to PH pathogenesis
  • BOLA3 can form a functional homodimer capable of engaging in Fe-S cluster transfer
  • BOLA3 is associated with adipose tissue oxidative capacity and it plays an indispensable role in beige adipocyte thermogenesis via maintaining mitochondrial homeostasis and adrenergic signaling-induced lipolysis
  • CELLULAR PROCESS cell cycle
    cell life, proliferation/growth
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • BOLA3 is potentially interacting with glutaredoxin 5, which along with several chaperones is involved in inserting [2Fe-2S] and [4Fe-4S] clusters into apoproteins
  • NFU1, BOLA3, ISCA2 and IBA57 operate in the maturation of [4Fe-4S] clusters, and IBA57 seems to require BOLA3, NFU1 and ISCA2 for its stability
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MMDS2
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional       loss of function
    inhibited thermogenesis activity without affecting lipogenesis in differentiated beige adipocytes
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS