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FLASH GENE
Symbol KCNH3 contributors: mct/npt/shn - updated : 09-12-2010
HGNC name potassium voltage-gated channel, subfamily H (eag-related), member 3
HGNC id 6252
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • PAS family, cytosolic N terminus domain, highly conserved, controlling desactivation by association with K+ channel
  • six membrane spanning domains
  • an hydrophobic pore
  • TM domains 5 and 6
  • arginine-rich fourth TM domain controling voltage dependent gating
  • cytosolic C terminus, sensitive to changes in membrane potential
  • C-terminal region containing a cyclic-nucleotide-binding homology domain (CNBHD) and C-linker that couples the CNBHD to the pore
    • HOMOLOGY
    interspecies ortholog to Kcnh3, Mus musculus
    ortholog to KCNH3, Pan troglodytes
    ortholog to Kcnh3, Rattus norvegicus
    Homologene
    FAMILY
  • potassium channel family
  • H (Eag) subfamily
    • CATEGORY transport channel
    SUBCELLULAR LOCALIZATION
    basic FUNCTION
  • may be involved in cellular excitability of restricted neurons in the human central nervous system
  • potassium voltage-gated channel
  • a potent regulator of excitability in hippocampal pyramidal neurons
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Kcnh3-/- mice exhibit neuronal hyperexcitability including frequent interictal spiking, spontaneous seizures and increased sensitivity to the chemoconvulsant pentylenetetrazol