KCNA1 Antibody (PACO49710)
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주문- SKU:
- PACO49710
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
Antibody Name: | KCNA1 Antibody (PACO49710) |
Antibody SKU: | PACO49710 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IF |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:500-1:5000, IF:1:50-1:200 |
Species Reactivity: | Human, Mouse, Rat |
Immunogen: | Recombinant Human Potassium voltage-gated channel subfamily A member 1 protein (7-150AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
Purification Method: | >95%, Protein G purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Western Blot. Positive WB detected in: Mouse brain tissue, Rat liver tissue. All lanes: KCNA1 antibody at 3µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 57 kDa. Observed band size: 57 kDa.. | |
Immunofluorescent analysis of HepG2 cells using PACO49710 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
Background: | Voltage-gated potassium channel that mediates transmembrane potassium transport in excitable membranes, primarily in the brain and the central nervous system, but also in the kidney. Contributes to the regulation of the membrane potential and nerve signaling, and prevents neuronal hyperexcitability. Forms tetrameric potassium-selective channels through which potassium ions pass in accordance with their electrochemical gradient. The channel alternates between opened and closed conformations in response to the voltage difference across the membrane. Can form functional homotetrameric channels and heterotetrameric channels that contain variable proportions of KCNA1, KCNA2, KCNA4, KCNA5, KCNA6, KCNA7, and possibly other family members as well; channel properties depend on the type of α subunits that are part of the channel. Channel properties are modulated by cytoplasmic β subunits that regulate the subcellular location of the α subunits and promote rapid inactivation of delayed rectifier potassium channels. In vivo, membranes probably contain a mixture of heteromeric potassium channel complexes, making it difficult to assign currents observed in intact tissues to any particular potassium channel family member. Homotetrameric KCNA1 forms a delayed-rectifier potassium channel that opens in response to membrane depolarization, followed by slow spontaneous channel closure. In contrast, a heterotetrameric channel formed by KCNA1 and KCNA4 shows rapid inactivation. Regulates neuronal excitability in hippocampus, especially in mossy fibers and medial perforant path axons, preventing neuronal hyperexcitability. Response to toxins that are selective for KCNA1, respectively for KCNA2, suggests that heteromeric potassium channels composed of both KCNA1 and KCNA2 play a role in pacemaking and regulate the output of deep cerebellar nuclear neurons (By similarity). May function as down-stream effector for G protein-coupled receptors and inhibit GABAergic inputs to basolateral amygdala neurons (By similarity). May contribute to the regulation of neurotransmitter release, such as gamma-aminobutyric acid, (GABA) release (By similarity). Plays a role in regulating the generation of action potentials and preventing hyperexcitability in myelinated axons of the vagus nerve, and thereby contributes to the regulation of heart contraction (By similarity). Required for normal neuromuscular responses. Regulates the frequency of neuronal action potential firing in response to mechanical stimuli, and plays a role in the perception of pain caused by mechanical stimuli, but does not play a role in the perception of pain due to heat stimuli (By similarity). Required for normal responses to auditory stimuli and precise location of sound sources, but not for sound perception (By similarity). The use of toxins that block specific channels suggest that it contributes to the regulation of the axonal release of the neurotransmitter dopamine (By similarity). Required for normal postnatal brain development and normal proliferation of neuronal precursor cells in the brain (By similarity). Plays a role in the reabsorption of Mg(2+) in the distal convoluted tubules in the kidney and in magnesium ion homeostasis, probably via its effect on the membrane potential. |
Synonyms: | Potassium voltage-gated channel subfamily A member 1 (Voltage-gated K(+) channel HuKI) (Voltage-gated potassium channel HBK1) (Voltage-gated potassium channel subunit Kv1.1), KCNA1 |
UniProt Protein Function: | 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. |
UniProt Protein Details: | Protein type:Channel, potassium; Membrane protein, multi-pass; Membrane protein, integral Chromosomal Location of Human Ortholog: 12p13.32 Cellular Component: voltage-gated potassium channel complex; cell surface; endoplasmic reticulum; integral to plasma membrane; cytoplasmic membrane-bound vesicle; dendrite; paranode region of axon; integral to membrane; perikaryon; cytosol; presynaptic membrane; cell soma; apical plasma membrane; plasma membrane; synapse; nerve terminal; cell junction Molecular Function:protein binding; voltage-gated potassium channel activity; potassium channel activity; delayed rectifier potassium channel activity; potassium ion transmembrane transporter activity Biological Process: detection of mechanical stimulus involved in sensory perception of pain; synaptic transmission; regulation of membrane potential; regulation of muscle contraction; neuromuscular process; generation of action potential; protein homooligomerization; potassium ion transport; detection of mechanical stimulus involved in sensory perception of touch Disease: Episodic Ataxia, Type 1 |
NCBI Summary: | 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] |
UniProt Code: | Q09470 |
NCBI GenInfo Identifier: | 119395748 |
NCBI Gene ID: | 3736 |
NCBI Accession: | NP_000208.2 |
UniProt Secondary Accession: | Q09470,Q3MIQ9, A6NM83, |
UniProt Related Accession: | Q09470 |
Molecular Weight: | 56,466 Da |
NCBI Full Name: | potassium voltage-gated channel subfamily A member 1 |
NCBI Synonym Full Names: | potassium voltage-gated channel, shaker-related subfamily, member 1 (episodic ataxia with myokymia) |
NCBI Official Symbol: | KCNA1 |
NCBI Official Synonym Symbols: | EA1; MK1; AEMK; HBK1; HUK1; MBK1; RBK1; KV1.1 |
NCBI Protein Information: | potassium voltage-gated channel subfamily A member 1; voltage-gated K(+) channel HuKI; voltage-gated potassium channel HBK1; voltage-gated potassium channel subunit Kv1.1 |
UniProt Protein Name: | Potassium voltage-gated channel subfamily A member 1 |
UniProt Synonym Protein Names: | Voltage-gated K(+) channel HuKI; Voltage-gated potassium channel HBK1; Voltage-gated potassium channel subunit Kv1.1 |
Protein Family: | Potassium voltage-gated channel subfamily |
UniProt Gene Name: | KCNA1 |
UniProt Entry Name: | KCNA1_HUMAN |