Calcium Channel, Voltage-Dependent, L Type, alpha 1S Subunit (CACNA1S) (N-Term) Antikörper

Antigen: Calcium Channel, Voltage-Dependent, L Type, alpha 1S Subunit (CACNA1S)

Synonyme: MHS5, HOKPP, Cav1.1, hypoPP, CCHL1A3, CACNL1A3, sj, fmd, mdg, Cchl1a3, AW493108, CACNA1S, caV1.1, cav1.1, ca(v)1.1

Epitope: N-Term

Klonalität: Polyklonal

Wirt: Kaninchen

Applikation: Immunhistochemie (IHC), Western Blot (WB)

Produktnummer: ABIN350148

Produktbeschreibung

Weitere Bezeichnung: CACNA1S (Cav1.1) (n-terminal)

Swiss-Prot: Q13698

Immunogen: A synthetic peptide from n-terminal of human CACNA1S (Cav1.1) conjugated to an immunogenic carrier protein was used as the immunogen. The antigen is homologous in rat and mouse.

Format: Lyophilized

Beschreibung: FUNCTION: Voltage-sensitive calcium channels (VSCC) mediate the entry of calcium ions into excitable cells and are also involved in a variety of calcium-dependent processes, including muscle contraction, hormone or neurotransmitter release, gene expression, cell motility, cell division and cell death. The isoform alpha-1S gives rise to L-type calcium currents. Long-lasting (L-type) calcium channels belong to the 'high-voltage activated' (HVA) group. They are blocked by dihydropyridines (DHP), phenylalkylamines, benzothiazepines, and by omega-agatoxin-IIIA (omega-Aga-IIIA). They are however insensitive to omega-conotoxin-GVIA (omega-CTx-GVIA) and omega-agatoxin-IVA (omega-Aga-IVA). Calcium channels containing the alpha-1S subunit play an important role in excitation-contraction coupling in skeletal muscle. SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein. Tissue specificity: Skeletal muscle specific. Defects in CACNA1S are a cause of periodic paralysis hypokalemic (HOKPP); also designated HYPOPP. HOKPP is an autosomal dominant disorder manifested by episodic flaccid generalized muscle weakness associated with falls of serum potassium levels. Defects in CACNA1S are the cause of malignant hyperthermia susceptibility 5 (MHS5); an autosomal dominant disorder that is potentially lethal in susceptible individuals on exposure to commonly used inhalational anesthetics and depolarizing muscle relaxants. Also known as: Voltage-dependent L-type calcium channel subunit alpha-1S, Voltage-gated calcium channel subunit alpha Cav1.1, Calcium channel, L type, alpha-1 polypeptide, isoform 3, skeletal muscle, CACH1, CACN1, CACNL1A3, CAC1S, Cav1.1.

Spezifität: Appears to be specific for CACNA1S.

Anwendungen

Applikationshinweise: IHC, WB. A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not tested in other applications.

Reinheit: whole serum

Lagerung: Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Publikationen

Publikationen: Hogan, Powers, Gregg: "Cloning of the human skeletal muscle alpha 1 subunit of the dihydropyridine-sensitive L-type calcium channel (CACNL1A3)." in: Genomics, Vol. 24, Issue 3, pp. 608-9, 1995 (PubMed).

Jurkat-Rott, Lehmann-Horn, Elbaz et al.: "A calcium channel mutation causing hypokalemic periodic paralysis." in: Human molecular genetics, Vol. 3, Issue 8, pp. 1415-9, 1995 (PubMed).

Gregory, Barlow, McLay et al.: "The DNA sequence and biological annotation of human chromosome 1." in: Nature, Vol. 441, Issue 7091, pp. 315-21, 2006 (PubMed).

Houinato, Laleye, Adjien et al.: "Hypokalaemic periodic paralysis due to the CACNA1S R1239H mutation in a large African family." in: Neuromuscular disorders : NMD, Vol. 17, Issue 5, pp. 419-22, 2007 (PubMed).

Matthews, Labrum, Sweeney et al.: "Voltage sensor charge loss accounts for most cases of hypokalemic periodic paralysis." in: Neurology, Vol. 72, Issue 18, pp. 1544-7, 2009 (PubMed).