蛋白名称
Potassium voltage-gated channel subfamily E member 3 (MinK-related peptide 2) (Minimum potassium ion channel-related peptide 2) (Potassium channel subunit beta MiRP2)
免疫原
Synthesized peptide derived from human protein . at AA range: 30-110
特异性
KCNE3 Monoclonal Antibody detects endogenous levels of protein.
组成
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
背景
potassium voltage-gated channel subfamily E regulatory subunit 3(KCNE3) Homo sapiens Voltage-gated potassium (Kv) channels represent the most complex class of voltage-gated ion channels from both functional and structural standpoints. Their diverse functions include regulating neurotransmitter release, heart rate, insulin secretion, neuronal excitability, epithelial electrolyte transport, smooth muscle contraction, and cell volume. This gene encodes a member of the potassium channel, voltage-gated, isk-related subfamily. This member is a type I membrane protein, and a beta subunit that assembles with a potassium channel alpha-subunit to modulate the gating kinetics and enhance stability of the multimeric complex. This gene is prominently expressed in the kidney. A missense mutation in this gene is associated with hypokalemic periodic paralysis. [provided by RefSeq, Jul 2008],
功能
disease:Defects in KCNE3 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 KCNE3 are a cause of thyrotoxic hypokalemic periodic paralysis (TPP) [MIM:188580]. TPP is seen in individuals of all races and manifests as attacks of episodic weakness with hypokalemia during thyrotoxicosis. TPP is seen most commonly in young Latin American or Asian men where up to 10% of thyrotoxic patients may have periodic paralysis. In such patients thyrotoxicosis has often been overlooked for many months. TPP generally occurs as a sporadic disease, and the periodic paralysis resolves completely with treatment of the thyrotoxicosis, although the muscle phenotype returns if the patient becomes thyrotoxic
