Potassium voltage-gated channel subfamily A member 1; Voltage-gated K(+) channel HuKI; Voltage-gated potassium channel HBK1; Voltage-gated potassium channel subunit Kv11; KCNA1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This KCNA1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 172-201 amino acids from the Central region of human KCNA1.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.3 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-KCNA1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This gene encodes a voltage-gated delayed potassium
channel that is phylogenetically related to the Drosophila Shaker
channel. The encoded protein has six putative transmembrane
segments (S1-S6), and the loop between S5 and S6 forms the pore and
contains the conserved selectivity filter motif (GYGD). The
functional channel is a homotetramer. The N-terminus of the channel
is associated with beta subunits that can modify the inactivation
properties of the channel as well as affect expression levels. The
C-terminus of the channel is complexed to a PDZ domain protein that
is responsible for channel targeting. Mutations in this gene have
been associated with myokymia with periodic ataxia (AEMK).

Neuroscience

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)

WB~~1:1000

KCNA1 Antibody (Center) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the KCNA1 antibody detected the KCNA1 protein (arrow).

KCNA1 Antibody (Center) (MBS9215239)immunohistochemistry analysis in formalin fixed and paraffin embedded human cerebellum tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of KCNA1 Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.

56466

potassium channel, voltage gated shaker related subfamily A, member 1

potassium voltage-gated channel subfamily A member 1

Potassium voltage-gated channel subfamily A member 1

KCNA1_HUMAN

This gene encodes a voltage-gated delayed potassium channel that is phylogenetically related to the Drosophila Shaker channel. The encoded protein has six putative transmembrane segments (S1-S6), and the loop between S5 and S6 forms the pore and contains the conserved selectivity filter motif (GYGD). The functional channel is a homotetramer. The N-terminus of the channel is associated with beta subunits that can modify the inactivation properties of the channel as well as affect expression levels. The C-terminus of the channel is complexed to a PDZ domain protein that is responsible for channel targeting. Mutations in this gene have been associated with myokymia with periodic ataxia (AEMK). [provided by RefSeq, Jul 2008]

Kv1.1: Mediates the voltage-dependent potassium ion permeability of excitable membranes. Assuming opened or closed conformations in response to the voltage difference across the membrane, the protein forms a potassium-selective channel through which potassium ions may pass in accordance with their electrochemical gradient. Defects in KCNA1 are the cause of episodic ataxia type 1 (EA1); also known as paroxysmal or episodic ataxia with myokymia (EAM) or paroxysmal ataxia with neuromyotonia. EA1 is an autosomal dominant disorder characterized by brief episodes of ataxia and dysarthria. Neurological examination during and between the attacks demonstrates spontaneous, repetitive discharges in the distal musculature (myokymia) that arise from peripheral nerve. Nystagmus is absent. Defects in KCNA1 are the cause of myokymia isolated type 1 (MK1). Myokymia is a condition characterized by spontaneous involuntary contraction of muscle fiber groups that can be observed as vermiform movement of the overlying skin. Electromyography typically shows continuous motor unit activity with spontaneous oligo- and multiplet-discharges of high intraburst frequency (myokymic discharges). Isolated spontaneous muscle twitches occur in many persons and have no grave significance. Belongs to the potassium channel family. A (Shaker) (TC 1.A.1.2) subfamily. Kv1.1/KCNA1 sub-subfamily.

Protein type: Membrane protein, multi-pass; Membrane protein, integral; Channel, potassium

Chromosomal Location of Human Ortholog: 12p13.32

Cellular Component: voltage-gated potassium channel complex; cell surface; endoplasmic reticulum; integral to plasma membrane; paranode region of axon; cytoplasmic membrane-bound vesicle; dendrite; integral to membrane; perikaryon; cytosol; presynaptic membrane; cell soma; apical plasma membrane; plasma membrane; synapse; nerve terminal; cell junction

Molecular Function: voltage-gated potassium channel activity; protein binding; potassium channel activity; delayed rectifier potassium channel activity; potassium ion transmembrane transporter activity

Biological Process: synaptic transmission; detection of mechanical stimulus involved in sensory perception of pain; regulation of membrane potential; regulation of muscle contraction; neuromuscular process; generation of action potential; potassium ion transport; protein homooligomerization; detection of mechanical stimulus involved in sensory perception of touch

Disease: Episodic Ataxia, Type 1

Graves, T.D., et al. Neurology 75(4):367-372(2010)
Bailey, S.D., et al. Diabetes Care (2010) In press :
van der Wijst, J., et al. J. Biol. Chem. 285(1):171-178(2010)
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
Kim, E., et al. Nature 378(6552):85-88(1995)

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