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FLASH GENE
Symbol KCNN3 contributors: mct/npt - updated : 20-06-2019
HGNC name potassium intermediate/small conductance calcium-activated channel, subfamily N, member 3
HGNC id 6292
Corresponding disease
ZLS3 Zimmerman-Laband Syndrome 3
Location 1q21.3      Physical location : 154.679.916 - 154.842.754
Synonym name small conductance calcium-activated potassium channel protein 3
Synonym symbol(s) SKCA3, SK3, KCa2.3, K3
DNA
TYPE functioning gene
STRUCTURE 162.85 kb     8 Exon(s)
Genomic sequence alignment details
10 Kb 5' upstream gene genomic sequence study
regulatory sequence Binding site
text structure two arrays of CAG trinucleotide repeats (polymorphism)binding sites for IK2, NKX25, NFAT, USF, CREB, BRN2, MZF1, VBP, MFM2 and several potential AP-1 and AP-4 sites
MAPPING cloned Y linked N status provisional
Physical map
LOC388701 1 LOC388701 NICE-3 1q21.2 NICE-3 protein NICE-4 1q22 NICE-4 protein HAX1 1q21 NICE-4 protein AQP10 1q21.2 aquaporin 10 ATP8B2 1q21.2 ATPase, Class I, type 8B, member 2 MRPS33P1 1q21.3 mitochondrial ribosomal protein S33 pseudogene 1 LOC388702 1 similar to 40S ribosomal protein SA (P40) (34/67 kDa laminin receptor) (Colon carcinoma laminin-binding protein) (NEM/1CHD4) (Multidrug resistance-associated protein MGr1-Ag) IL6R 1q21 interleukin 6 receptor LOC388703 1 similar to hypothetical protein 9430022A14 DKFZp434M202 1q22 hypothetical protein DKFZp434M202 UBE2Q 1q22 ubiquitin-conjugating enzyme E2Q (putative) CHRNB2 1q21.3 cholinergic receptor, nicotinic, beta polypeptide 2 (neuronal) ADAR 1q21.1-q21.2 adenosine deaminase, RNA-specific KCNN3 1q21.3 potassium intermediate/small conductance calcium-activated channel, subfamily N, member 3 PMVK 1p13-q23 phosphomevalonate kinase PBXIP1 1q22 pre-B-cell leukemia transcription factor interacting protein 1 PYGO2 1q22 pygopus 2 SHC1 1q21 SHC (Src homology 2 domain containing) transforming protein 1 CKS1B 1q21.2 CDC28 protein kinase regulatory subunit 1B PP591 1q22 FAD-synthetase LENEP 1q22 lens epithelial protein ZFP67 1q21.2 zinc finger protein 67 homolog (mouse) FLJ32934 1q22 hypothetical protein FLJ32934 FLJ32785 1q22 hypothetical protein FLJ32785 ADAM15 1q21.3 a disintegrin and metalloproteinase domain 15 (metargidin)
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
8 - 3060 - 731 - Wittekindt (1998)
8 - 1981 - 426 - Wittekindt (1998)
EXPRESSION
Type
constitutive of
   expressed in (based on citations)
organ(s)
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Nervouscentral   
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • intracellular N and C termini
  • six highly conserved transmembrane segments
  • two arrays of polyglutamine (CAG trinucleotide) repeats in the N terminal region
  • mono polymer heteromer , octamer
    HOMOLOGY
    interspecies homolog to murine Kcnn3
    Homologene
    FAMILY
  • potassium channel KCNN family
  • CATEGORY transport channel
    SUBCELLULAR LOCALIZATION     plasma membrane
    basic FUNCTION
  • playing a role in determining the firing pattern of neurons
  • forming a voltage-independent potassium channel activated by intracellular calcium
  • potassium channel protein involved in atrial repolarization (Ellinor 2010)
  • KCNN3 channel might be the only member of the Ca(2+)-activated K(+) channel family involved in melanoma cell motility pathways (Chantome 2009)
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS development
    text
  • neurogenesis
  • potassium transport
  • PATHWAY
    metabolism
    signaling neurotransmission
    synaptic transmission
    a component
  • heterooligomer complex composed of four channel subunits, each of which binds to a calmodulin subunit regulating the channel activity through calcium-binding
  • ABI1 and the neural WASL, forms a complex with KCNN3- channels in neural stem cells, involved in early neurogenesis
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • KCNN3 and calmodulin subunit
  • cell & other
    REGULATION
    activated by Ca2+
    inhibited by apamin
    ASSOCIATED DISORDERS
    corresponding disease(s) ZLS3
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --over  
    has a critical role in the increased Ca(2+)-induced fragility in DM1 cells, leading to impaired growth through a greater calcium-induced fragility (Rhodes 2006)
    Susceptibility
  • to anorexia nervosa
  • to juvenile myoclonic epilepsy
  • to atrial fibrillation (AF)
  • Variant & Polymorphism SNP , repeat
  • polymorphic CAG repeat (19 to 28 repeats)contributor to anorexia nervosa
  • polymorphic CAG repeat (16 to 19 repeats) increase susceptibility to juvenile myoclonic epilepsy
  • SNP acting as genetic modifier of the cardiac phenotype in myotonic dystrophy type 1 patient (Rinaldi 2008)
  • Candidate gene
    Marker
    Therapy target potential direct or indirect target for the pharmacological treatment of AF may also merit further investigation (Ellinor 2010)
    ANIMAL & CELL MODELS
    absence or overexpression of KCNN3 (Bond,2000)