Mouse anti-Human, Mouse SCN8A Monoclonal Antibody | anti-SCN8A antibody

SCN8A (Sodium Channel Protein Type 8 Subunit alpha, Sodium Channel Protein Type VIII Subunit alpha, Voltage-gated Sodium Channel Subunit alpha Nav1.6, MED) (Biotin)

Cat. Num. AAA6144238

• Gene Names: SCN8A; MED; PN4; CIAT; BFIS5; NaCh6; CERIII; EIEE13; Nav1.6
• Reactivity: Human, Mouse
• Applications: ELISA, Immunofluorescence, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: SCN8A; Monoclonal Antibody; SCN8A (Sodium Channel Protein Type 8 Subunit alpha; Sodium Channel Protein Type VIII Subunit alpha; Voltage-gated Sodium Channel Subunit alpha Nav1.6; MED) (Biotin); anti-SCN8A antibody

• Host: Mouse
• Reactivity: Human, Mouse
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 4G7
• Specificity: Recognizes human SCN8A. Species Crossreactivity: mouse.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-SCN8A antibody: ELISA (EIA), Immunofluorescence (IF), Western Blot (WB)
• Application Notes: IF: 10ug/ml; Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa1854-1952 from human SCN8A (NP_055006) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: RVLGDSGELDILRQQMEERFVASNPSKVSYEPITTTLRRKQEEVSAVVLQRAYRGHLARRGFICKKTTSNKLENGGTHREKKESTPSTASLPSYDSVT*
• Conjugate: Biotin
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (36.89kD).)

Western Blot (WB)

(SCN8A monoclonal antibody, Western Blot analysis of SCN8A expression in NIH/3T3.)

Immunofluorescence (IF)

(Immunofluorescence of monoclonal antibody to SCN8A on NIH/3T3 cell. [antibody concentration 10ug/ml].)

Product Categories/Family for anti-SCN8A antibody

Antibodies; Ion Channel

References

1. Na(v)1. 1 localizes to axons of parvalbumin-positive inhibitory interneurons: a circuit basis for epileptic seizures in mice carrying an Scn1a gene mutation. Ogiwara I, Miyamoto H, Morita N, Atapour N, Mazaki E, Inoue I, Takeuchi T, Itohara S, Yanagawa Y, Obata K, Furuichi T, Hensch TK, Yamakawa K.J Neurosci. 2007 May 30;27(22):5903-14.

NCBI and Uniprot Product Information

• NCBI GI #: 7657544
• NCBI GeneID: 6334
• NCBI Accession #: NP_055006
• NCBI GenBank Nucleotide #: NM_014191
• UniProt Accession #: Q9UQD0
• Molecular Weight: 225kDa
• NCBI Official Full Name: sodium channel protein type 8 subunit alpha isoform 1
• NCBI Official Synonym Full Names: sodium voltage-gated channel alpha subunit 8
• NCBI Official Symbol: SCN8A
• NCBI Official Synonym Symbols: MED; PN4; CIAT; BFIS5; NaCh6; CERIII; EIEE13; Nav1.6
• NCBI Protein Information: sodium channel protein type 8 subunit alpha
• UniProt Protein Name: Sodium channel protein type 8 subunit alpha
• Protein Family: Sodium channel protein
• UniProt Gene Name: SCN8A
• UniProt Synonym Gene Names: MED
• UniProt Entry Name: SCN8A_HUMAN

NCBI Description

This gene encodes a member of the sodium channel alpha subunit gene family. The encoded protein forms the ion pore region of the voltage-gated sodium channel. This protein is essential for the rapid membrane depolarization that occurs during the formation of the action potential in excitable neurons. Mutations in this gene are associated with cognitive disability, pancerebellar atrophy and ataxia. Alternate splicing results in multiple transcript variants.[provided by RefSeq, May 2010]

Uniprot Description

SCN8A: Mediates the voltage-dependent sodium ion permeability of excitable membranes. Assuming opened or closed conformations in response to the voltage difference across the membrane, the protein forms a sodium-selective channel through which Na(+) ions may pass in accordance with their electrochemical gradient. In macrophages and melanoma cells, isoform 5 may participate in the control of podosome and invadopodia formation. Defects in SCN8A are the cause of cognitive impairment with or without cerebellar ataxia (CIAT). A disorder characterized by markedly delayed cognitive and motor development, attention deficit disorder, and cerebellar ataxia. Features include bilateral esophoria, strabismatic amblyopia, unsustained gaze evoked nystagmus on horizontal gaze, ataxic gait, dysmetria in the upper limbs and dysarthria, with normal strength, tone, and reflexes. Defects in SCN8A are the cause of epileptic encephalopathy, early infantile, type 13 (EIEE13). A form of epilepsy characterized by frequent tonic seizures or spasms beginning in infancy with a specific EEG finding of suppression-burst patterns, characterized by high-voltage bursts alternating with almost flat suppression phases. Patients may progress to West syndrome, which is characterized by tonic spasms with clustering, arrest of psychomotor development, and hypsarrhythmia on EEG. EIEE13 is a severe form consisting of early-onset seizures, features of autism, intellectual disability, ataxia, and sudden unexplained death in epilepsy. Belongs to the sodium channel (TC 1.A.1.10) family. Nav1.6/SCN8A subfamily. 5 isoforms of the human protein are produced by alternative splicing.

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

Chromosomal Location of Human Ortholog: 12q13

Cellular Component: voltage-gated sodium channel complex; cell soma; cytoplasmic membrane-bound vesicle; dendrite; integral to membrane; plasma membrane; Z disc

Molecular Function: voltage-gated sodium channel activity; ATP binding

Biological Process: myelination; nervous system development; muscle development; sensory perception of sound; response to toxin; neuromuscular process; sodium ion transport; generation of action potential; adult walking behavior; peripheral nervous system development

Disease: Epileptic Encephalopathy, Early Infantile, 13; Cognitive Impairment With Or Without Cerebellar Ataxia