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HRPBiotinGoldRBITCAPFITCCy3Cy5Cy5.5Cy7PEPE-Cy3PE-Cy5PE-Cy5.5PE-Cy7APCAlexa Fluor 350Alexa Fluor 488Alexa Fluor 555Alexa Fluor 594Alexa Fluor 647

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HRPBiotinGoldRBITCAPFITCCy3Cy5Cy5.5Cy7PEPE-Cy3PE-Cy5PE-Cy5.5PE-Cy7APCAlexa Fluor 350Alexa Fluor 488Alexa Fluor 555Alexa Fluor 594Alexa Fluor 647

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Anti-DHPR alpha 1/Cy7

货号: bs-2806R-Cy7 基本售价: 2980.0 元 规格: 100ul

产品信息

产品编号
bs-2806R-Cy7
英文名称
Anti-DHPR alpha 1/Cy7
中文名称
Cy7标记的L-型电压依赖型钙通道α抗体
别    名
alpha-1 polypeptide; cardiac muscle; isoform 1; L type; CAC1C_HUMAN; CACH 2; DHPR alpha 1; VDCC-L alpha; CACH2; CACN 2; CACN2; CACNA1C; CACNL1A1; Calcium channel; Calcium channel cardic dihydropyridine sensitive alpha 1 subunit; Calcium channel L type alpha 1 polypeptide isoform 1 cardiac muscle; Calcium channel voltage dependent L type alpha 1C subunit; CaV1.2; CCHL1A1; DHPR alpha 1 subunit; LQT8; TS; Voltage dependent L type calcium channel alpha 1C subunit; Voltage dependent L type calcium channel subunit alpha 1C; Voltage gated calcium channel alpha subunit Cav1.2; Voltage gated calcium channel subunit alpha Cav1.2; Voltage gated L type calcium channel Cav1.2 alpha 1 subunit, splice variant 10*; Voltage-dependent L-type calcium channel subunit alpha-1C; Voltage-gated calcium channel subunit alpha Cav1.2.  
规格价格
100ul/2980元购买        大包装/询价
说 明 书
100ul  
研究领域
心血管  免疫学  神经生物学  通道蛋白  
抗体来源
Rabbit
克隆类型
Polyclonal
交叉反应
Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, 
产品应用

not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量
249kDa
性    状
Lyophilized or Liquid
浓    度
1mg/ml
免 疫 原
KLH conjugated synthetic peptide derived from human DHPR alpha 1
亚    型
IgG
纯化方法
affinity purified by Protein A
储 存 液
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍
background:
This gene encodes an alpha-1 subunit of a voltage-dependent calcium channel. Calcium channels mediate the influx of calcium ions into the cell upon membrane polarization. The alpha-1 subunit consists of 24 transmembrane segments and forms the pore through which ions pass into the cell. The calcium channel consists of a complex of alpha-1, alpha-2/delta, beta, and gamma subunits in a 1:1:1:1 ratio. There are multiple isoforms of each of these proteins, either encoded by different genes or the result of alternative splicing of transcripts. The protein encoded by this gene binds to and is inhibited by dihydropyridine. Alternative splicing results in many transcript variants encoding different proteins. [provided by RefSeq]

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 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. Interacts (via C-terminal CDB motif) with CABP5; in a calcium-dependent manner.

Subcellular Location:
Membrane; Multi-pass membrane protein. Cell membrane. Note=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.

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.

L型钙通道广泛分布于心肌细胞膜上,尤其T管上含量最为丰富,是心肌细胞膜的主要钙通道类型。L型钙通道的开闭主要受膜电位变化的影响,是电压依赖性钙通道,激活电位-40~-30mV,失活电位-20mV。L型钙通道开放后持续的时间长较长,激活占时20~30ms,失活更慢(100~300ms),又称为慢钙通道。