Molecular weight (DA)
200kD
Immunogen
The antiserum was produced against synthesized peptide derived from human SCN4A. AA range:431-480
Specificity
Na+ CP type IVα Polyclonal Antibody detects endogenous levels of Na+ CP type IVα protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB 1:500-2000;IHC-p 1:50-300
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
Voltage-gated sodium channels are transmembrane glycoprotein complexes composed of a large alpha subunit with 24 transmembrane domains and one or more regulatory beta subunits. They are responsible for the generation and propagation of action potentials in neurons and muscle. This gene encodes one member of the sodium channel alpha subunit gene family. It is expressed in skeletal muscle, and mutations in this gene have been linked to several myotonia and periodic paralysis disorders. [provided by RefSeq, Jul 2008],
Function
disease:Defects in SCN4A are a cause of periodic paralysis hypokalemic (HOKPP) [MIM:170400]; also designated HYPOPP. HOKPP is an autosomal dominant disorder manifested by episodic flaccid generalized muscle weakness associated with falls of serum potassium levels.,disease:Defects in SCN4A are the cause of a congenital myasthenic syndrome due to mutation in SCNA4 (CMSSCNA4) [MIM:603967]. CMSSCNA4 is a congenital myasthenic syndrome associated with fatigable generalized weakness and recurrent attacks of respiratory and bulbar paralysis since birth. The fatigable weakness involves lid-elevator, external ocular, facial, limb and truncal muscles and an decremental response of the compound muscle action potential on repetitive stimulation.,disease:Defects in SCN4A are the cause of myotonia SCN4A-related (MYOSCN4A) [MIM:608390]. Myotonia is characterized by sustained muscle tensing that prevent
Protein name
Sodium channel protein type 4 subunit alpha
Abbreviation
Na+ CP type IVα
Other name
SCN4A; Sodium channel protein type 4 subunit alpha; SkM1; Sodium channel protein skeletal muscle subunit alpha; Sodium channel protein type IV subunit alpha; Voltage-gated sodium channel subunit alpha Nav1.4
Human protein sequence Database
P35499
Mouse protein sequence database
Q9ER60
Rat protein sequence database
P15390
Cellular localization
Cell membrane ; Multi-pass membrane protein .
Tissue expression
Skeletal muscle,
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