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FLASH GENE
Symbol SURF1 contributors: mct/ - updated : 05-12-2016
HGNC name surfeit 1
HGNC id 11474
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • an aminoterminal characteristic mitochondrial targeting sequence
  • N and C termini transmembrane segments
  • a central loop spanning the intermembrane space
    • mono polymer heteromer , complex
    HOMOLOGY
    interspecies homolog to yeast SHY1(mitochondrial protein)
    ortholog to murine Surf1
    homolog to Drosophila Surf1
    homolog to C.elegans H06I04.2
    Homologene
    FAMILY
  • SURF1 family
    • CATEGORY enzyme , transport carrier
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,membrane
    text anchored to the inner mitochondrial membrane by two transmembrane domains
    basic FUNCTION
  • involved in the biogenesis of an active cytochrome c oxidase complex
  • having a direct role in heme a cofactor insertion into COX subunit I by providing a protein-bound heme a pool
  • appears to be involved in the early step of COX assembly
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS respiration , electron transport
    text aerobic respiration
    PATHWAY
    metabolism
    signaling
    a component
  • associated with a primitive quinol oxidase belonging to the cytochrome oxidase
    • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • is a specific assembly factor of cytochrome c oxidase (COX)
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) SNELS , CMT4K
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • SURF1(-/-) mouse fibroblasts were characterized by low content of COX1 assembly intermediates and milder decrease in COX monomer, which appeared more stable
  • mice carrying a homozygous knockout (KO) of the Surf1 gene showed a significant decrease in mitochondrial electron transport chain Complex IV activity, yet displayed increased lifespan and reduced brain damage after excitotoxic insults