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Rabbit Anti-CACNA1S/BF647 Conjugated antibody (bs-9925R-BF647)
訂購(gòu)熱線(xiàn):400-901-9800
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訂購(gòu)QQ:  400-901-9800
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-9925R-BF647
英文名稱(chēng) Rabbit Anti-CACNA1S/BF647 Conjugated antibody
中文名稱(chēng) BF647標(biāo)記的電壓依賴(lài)性鈣通道Cav1.1抗體
別    名 L-type Ca++ CP α1S: alpha-1 polypeptide; isoform 3; L type; skeletal muscle; CAC1S_HUMAN; CACH1; Cach1b; CACN1; CACNA1S; CACNL1A3; Calcium channel; Calcium channel, L type, alpha 1 polypeptide, isoform 3, skeletal; Calcium channel, L type, alpha 1 polypeptide, isoform 3, skeletal muscle; Calcium channel, L type, alpha 1 polypeptide, isoform 3, skeletal muscle, hypokalemic periodic paralysis; Calcium channel, skeletal muscle dihydropyridine sensitive , alpha 1 subunit; Calcium channel, voltage dependent, L type, alpha 1S subunit; Calcium channel, voltage-dependent, L type, alpha 1S subunit, b; Cav1.1; CCHL1A3. Dihydropyridine receptor; Dihydropyridine sensitive L type calcium channel alpha 1 subunit; fmd; HOKPP; HypoPP; Malignant hyperthermia susceptibility 5; mdg; MHS5; ROB1; sj; TTPP1; Voltage gated calcium channel subunit alpha Cav1.1; Voltage-dependent L-type calcium channel subunit alpha-1S; Voltage-gated calcium channel subunit alpha Cav1.1  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Pig, Cow, Rabbit, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 212kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CACNA1S
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
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.

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.

Subunit:
Multisubunit complex 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. An additional gamma subunit is present only in skeletal muscle L-type channel. Interacts with DYSF and JSRP1. Interacts with RYR1 (By similarity).

Subcellular Location:
Membrane.

Tissue Specificity:
Skeletal muscle specific.

Post-translational modifications:
Phosphorylation by PKA activates the calcium channel (By similarity).

DISEASE:
Defects in CACNA1S are the cause of periodic paralysis hypokalemic type 1 (HOKPP1) [MIM:170400]; also designated HYPOPP. HOKPP1 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 type 5 (MHS5) [MIM:601887]; an autosomal dominant disorder that is potentially lethal in susceptible individuals on exposure to commonly used inhalational anesthetics and depolarizing muscle relaxants.
Defects in CACNA1S are the cause of susceptibility to thyrotoxic periodic paralysis type 1 (TTPP1) [MIM:188580]. A sporadic muscular disorder characterized by episodic weakness and hypokalemia during a thyrotoxic state. It is clinically similar to hereditary hypokalemic periodic paralysis, except for the fact that hyperthyroidism is an absolute requirement for disease manifestation. The disease presents with recurrent episodes of acute muscular weakness of the four extremities that vary in severity from paresis to complete paralysis. Attacks are triggered by ingestion of a high carbohydrate load or strenuous physical activity followed by a period of rest. Thyrotoxic periodic paralysis can occur in association with any cause of hyperthyroidism, but is most commonly associated with Graves disease.

Similarity:
Belongs to the calcium channel alpha-1 subunit (TC 1.A.1.11) family. CACNA1S subfamily.

Database links:

Entrez Gene: 779 Human

Entrez Gene: 12292 Mouse

Entrez Gene: 100009585 Rabbit

Entrez Gene: 682930 Rat

Omim: 114208 Human

SwissProt: Q13698 Human

SwissProt: Q02789 Mouse

SwissProt: P07293 Rabbit

SwissProt: Q02485 Rat

Unigene: 1294 Human

Unigene: 4418 Mouse

Unigene: 10738 Rat

Unigene: 220579 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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