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FLASH GENE
Symbol SSPN contributors: mct - updated : 04-03-2015
HGNC name sarcospan (Kras oncogene-associated gene)
HGNC id 11322
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • four transmembrane segments
  • cytoplasmic N and C termini
    • HOMOLOGY
    Homologene
    FAMILY
  • dystrophin-glycoprotein complex (DGC) family
    • CATEGORY structural protein , protooncogene
    SUBCELLULAR LOCALIZATION     plasma membrane,junction
    text sarcolemma
    basic FUNCTION
  • stabilizing the link between dystroglycan and dystrophin/utrophin
  • negatively regulates RNAPII transcriptional activity by inhibiting recruitment of Swi/Snf to the CYC1 promoter, and this inhibition is abrogated by the SSPN-SUPT6H interaction (Zhang 2008)
  • having functions to the transition from an inactive to an actively transcribing RNAPII complex at a postrecruitment-regulated promoter (Zhang 2008)
  • stabilizes the sarcolemma by increasing levels of the utrophin-glycoprotein complex (UGC) at the extrasynaptic membrane to compensate for the loss of dystrophin
  • SSPN-dependent AKT1 activation is required for UTRN expression and muscle regeneration
  • SSPN is a necessary component of DMD and UTRN function and SSPN modulation of integrin signaling is required for extracellular matrix attachment and muscle force development
  • important regulator of muscle cell adhesion, strength, and regeneration
  • regulates glycosylation of a specific subpopulation of alpha-dystroglycan, the laminin-binding receptor associated with dystrophin and utrophin, localized to the neuromuscular junction
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • complexing with sarcoglycans in a stoichiometrically equal complex
  • core component of the major adhesion complexes in skeletal muscle, the dystrophin- and utrophin (Utr)-glycoprotein complexes (DGC and UGC)
    • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • dystrophin associated
  • interaction with SUP6TH (SSPN is recruited via interaction with RNAPII, and is necessary for SUP6TH recruitment) (Zhang 2008)
  • SSPN directly interacts with the utrophin-glycoprotein complex (UGC) and functions to stabilize UTRN without increasing UTRN transcription (pMID: 18981229)
  • SSPN regulates AKT1 signaling to control UTRN expression
  • interactions between integrin and SSPN are critical for maintenance of the dystrophin-glycoprotein complex and force production of the diaphragm muscle
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    ANIMAL & CELL MODELS