Recombinant人CACNA1S protein (ab114520)

概述

  • 产品名称Recombinant人CACNA1S protein
  • 蛋白长度Protein fragment

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionQ13698
    • 种属Human
    • 序列CPRVESSMPEDRKSSTPGSLHEETPHSRSTRENTSRCSAPATALLIQKAL VRGGLGTLAADANFIMATGQALGDACQMEPEEVEIMATELLKGREA
    • 分子量36 kDa including tags
    • 氨基酸1743 to 1838

技术指标

Our Abpromise guarantee covers the use of ab114520 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    SDS-PAGE

    ELISA

    Western blot

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.79% Tris HCl, 0.3% Glutathione

常规信息

  • 别名
    • alpha-1 polypeptide
    • 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
    • isoform 3
    • L type
    • Malignant hyperthermia susceptibility 5
    • mdg
    • MHS5
    • ROB1
    • sj
    • skeletal muscle
    • 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
    see all
  • 功能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.
  • 组织特异性Skeletal muscle specific.
  • 疾病相关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.
  • 序列相似性Belongs to the calcium channel alpha-1 subunit (TC 1.A.1.11) family. CACNA1S subfamily.
  • 结构域Each of the four internal repeats contains five hydrophobic transmembrane segments (S1, S2, S3, S5, S6) and one positively charged transmembrane segment (S4). S4 segments probably represent the voltage-sensor and are characterized by a series of positively charged amino acids at every third position.
    The loop between repeats II and III interacts with the ryanodine receptor, and is therefore important for calcium release from the endoplasmic reticulum necessary for muscle contraction.
  • 翻译后修饰Phosphorylation by PKA activates the calcium channel.
  • 细胞定位Membrane.
  • Information by UniProt

Recombinant Human CACNA1S protein 图像

  • 12.5% SDS-PAGE analysis of CACNA1S protein (ab114520). Stained with Coomassie Blue.

Recombinant Human CACNA1S protein (ab114520)参考文献

ab114520 has not yet been referenced specifically in any publications.

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