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BSCL2 Rabbit pAb(bs-6311R)-50u

l/100ul/200ul
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  • ¥1180 - 2800
  • Bioss已认证
  • bs-6311R
  • 2025年10月24日
  • 产品信息以Bioss网站为准
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      50ul/100ul/200ul

    规格:50ul产品价格:¥1180.0
    规格:100ul产品价格:¥1980.0
    规格:200ul产品价格:¥2800.0
    产品编号bs-6311R
    英文名称BSCL2 Rabbit pAb
    中文名称先天性脂肪代谢障碍蛋白2抗体(常染色体显性遗传痉挛性截瘫17)
    英文别名Bernardinelli Seip congenital lipodystrophy 2; Bernardinelli Seip congenital lipodystrophy type 2 protein; Bernardinelli-Seip congenital lipodystrophy type 2 protein; BSCL 2; BSCL2; BSCL2_HUMAN; GNG3LG; HMN 5; HMN5; MGC4694; Seipin; Spastic paraplegia 17(autosomal dominant); Spastic paraplegia 17(Silver syndrome); Spastic paraplegia 17; Spastic paraplegia with amyotrophy of hands and feet(Silver syndrome); Spastic paraplegia with amyotrophy of hands and feet; SPG 17; SPG17.
    产品应用IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500

    Not yet tested in other applications.
    Optimal working dilutions must be determined by the end user.

    交叉反应Rat (Human, Mouse, Dog, Cow, Horse, Rabbit)
    抗体来源Rabbit
    免疫原KLH conjugated synthetic peptide derived from human BSCL2/SPG17
    亚型IgG
    性状Liquid
    纯化方法affinity purified by Protein A
    克隆类型Polyclonal
    理论分子量44 kDa
    浓度1mg/ml
    储存液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    研究领域

    Cardiovascular > Atherosclerosis > Diabetes associated

    Metabolism > Types of disease > Obesity

    Neuroscience > Neurology process > Neurodegenerative disease

    Tags & Cell Markers > Subcellular Markers > Organelles > ER

    组织特异性Highest expression in brain and testis.
    相似性Belongs to the seipin family.
    功能Endoplasmic reticulum membrane; Multi-pass membrane protein.
    保存条件Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    背景资料Defects in BSCL2 are the cause of congenital generalized lipodystrophy type 2 (CGL2) . Congenital generalized lipodystrophy is an autosomal recessive disorder characterized by a near absence of adipose tissue, extreme insulin resistance, hypertriglyceridemia, hepatic steatosis and early onset of diabetes.Defects in BSCL2 are the cause of spastic paraplegia type 17 (SPG17) ; also known as Silver spastic paraplegia syndrome. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. SPG17 is characterized by prominent amyotrophy of the hand muscles, the presence of mild to severe pyramidal tract signs, and spastic paraplegia. SPG17 is a motor neuron disease overlapping with distal spinal muscular atrophy type 5.Defects in BSCL2 are a cause of distal hereditary motor neuropathy type 5 (HMN5); also known aS distal hereditary motor neuropathy type V (DSMAV). HMN5 is an autosomal dominant disorder characterized by degeneration of motor nerve fibers, predominantly in limb distal regions.

     

    应用推荐稀释比例
    {IHC-P}{1:100-500}
    {IHC-F}{1:100-500}
    {IF}{1:100-500}

     

    产品细节图片1
    Tissue/cell: rat brain tissue; 4% P‌‌araformaldehyde-fixed and paraffin-embedded;
    Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
    Incubation: Anti-BSCL2 Polyclonal Antibody, Unconjugated(bs-6311R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining

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    BSCL2 Rabbit pAb(bs-6311R)-50ul/100ul/200ul
    ¥1180 - 2800