L型钙通道蛋白抗体
规格：1mg/1ml
英文名： CACH2/CACNA1C
别名： CACH 2;CACH2; CACN 2; CACN2; CACNA1C; CACNL1A1; Calcium channel cardic dihydropyridine sensitive alpha 1 subunit; Calcium channel L type alpha 1 polypeptide isoform 1 cardiac muscle; Calcium channel v
分子量： 239kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human CACH2
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
细胞定位：细胞膜
L型钙通道蛋白抗体产品介绍：background: The receptor for the 1,4-dihydropyrodine (DHPR) class of Ca2+ channel is most abundant in the transverse tubular membranes of skeletal muscle. DHRP is essential in excitation-contraction (E-C) coupling and has been proposed to have a dual function as a calcium channel and voltage sensor. Skeletal muscle DHRP consists of four subunits: alpha1 (170kDa); alpha2 (175kDa non-reduced, 150kDa reduced); beta (52kDa) and gamma (32kDa). 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-1C 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-1C subunit play an important role in excitation-contraction coupling in the heart. The various isoforms display marked differences in the sensitivity to DHP compounds. Binding of calmodulin or CABP1 at the same regulatory sites results L型钙通道蛋白抗体in an opposit effects on the channel function. Subunit: Voltage-dependent calcium channels are multisubunit complexes, consisting of alpha-1, alpha-2, beta and delta subunits in a 1:1:1:1 ratio. The channel activity is directed by the pore-forming and voltage-sensitive alpha-1 subunit. In many cases, this subunit is sufficient to generate voltage-sensitive calcium channel activity. The auxiliary subunits beta and alpha-2/delta linked by a disulfide bridge regulate the channel activity. Interacts with CACNA2D4. Interacts (via the N-terminus and the C-terminal C and IQ motifs) with CABP1. The binding via the C motif is calcium independent whereas the binding via IQ requires the presence of calcium and is mutually exclusive with calmodulin binding. The binding to the cytoplasmic N-terminal domain is calcium independent but is essential for the channel modulation. L型钙通道蛋白抗体Interacts (via C-terminal CDB motif) with CABP5; in a calcium-dependent manner (By similarity). Subcellular Location: Membrane. Cell membrane. The interaction between RRAD and CACNB2 regulates its trafficking to the cell membrane. Tissue Specificity: Expressed in brain, heart, jejunum, ovary, pancreatic beta-cells and vascular smooth muscle. Overall expression is reduced in atherosclerotic vascular smooth muscle. Post-translational modifications: Phosphorylation by PKA activates the channel. DISEASE: Defects in CACNA1C are the cause of Timothy syndrome (TS) [MIM:601005]. TS is a disorder characterized by multiorgan dysfunction including lethal arrhythmias, webbing of fingers and toes, congenital heart disease, immune deficiency, intermittent hypoglycemia, cognitive abnormalities and autism. Defects in CACNA1C are the cause of Brugada syndrome type 3 (BRGDA3) [MIM:611875]. A heart disease characterized by the association of Brugada syndrome with shortened QT intervals. Brugada syndrome is a tachyarrhythmia characterized by right bundle branch block and ST segment elevation on an electrocardiogram (ECG). It can cause the ventricles to beat so fast that the blood is prevented from circulating efficiently in the body. When this situation occurs (called ventricular fibrillation), the individual will faint and may die in a few minutes if the heart is not reset. Similarity: Belongs to the calcium channel alpha-1 subunit (TC 1.A.1.11) family. CACNA1C subfamily. Gene ID: 775 Database links: Entrez Gene: 775 Human Entrez Gene: 12288 Mouse Entrez Gene: 100144322 Rabbit Entrez Gene: 24239 Rat Omim: 114205 Human SwissProt: Q13936 Human SwissProt: Q01815 Mouse SwissProt: P15381 Rabbit SwissProt: P22002 Rat Unigene: 118262 Human Unigene: 436656 Mouse Unigene: 9827 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. Involvement in disease; Defects in CACNA1C are the cause of Timothy syndrome (TS) . TS is a disorder characterized by multiorgan dysfunction including lethal arrhythmias, webbing of fingers and toes, congenital heart disease, immune deficiency, intermittent hypoglycemia, cognitive abnormalities and autism. Defects in CACNA1C are the cause of Brugada syndrome type 3 (BRGDA3). A heart disease characterized by the association of Brugada syndrome with shortened QT intervals. Brugada syndrome is a tachyarrhythmia characterized by right bundle branch block and ST segment elevation on an electrocardiogram (ECG). It can cause the ventricles to beat so fast that the blood is prevented from circulating efficiently in the body. When this situation occurs (called ventricular fibrillation), the individual will faint and may die in a few minutes if the heart is not reset.
L型钙通道蛋白抗体产品应用：ELISA=1:1000-5000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 神经生物学 信号转导 通道蛋白
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid