製品の概要

  • 製品名Anti-BSCL2 antibody
    BSCL2 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to BSCL2
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Synthetic peptide corresponding to a region within N terminal amino acids 85-115 of Human BSCL2 (NP_001116427.1, NP_001124174.1, NP_116056.3), conjugated to KLH.

  • ポジティブ・コントロール
    • NCI-H460 cell line lysates

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab107170 in the following tested applications.

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

アプリケーション Abreviews 特記事項
WB 1/100 - 1/500. Predicted molecular weight: 44 kDa.

ターゲット情報

  • 組織特異性Highest expression in brain and testis.
  • 関連疾患Defects in BSCL2 are the cause of congenital generalized lipodystrophy type 2 (CGL2) [MIM:269700]. 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) [MIM:270685]; 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) [MIM:600794]; 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.
  • 配列類似性Belongs to the seipin family.
  • 細胞内局在Endoplasmic reticulum membrane.
  • Information by UniProt
  • 参照データベース
  • 別名
    • Bernardinelli Seip congenital lipodystrophy 2 antibody
    • Bernardinelli Seip congenital lipodystrophy type 2 protein antibody
    • Bernardinelli-Seip congenital lipodystrophy type 2 protein antibody
    • BSCL 2 antibody
    • BSCL2 antibody
    • BSCL2_HUMAN antibody
    • GNG3LG antibody
    • HMN 5 antibody
    • HMN5 antibody
    • MGC4694 antibody
    • Seipin antibody
    • Spastic paraplegia 17 (autosomal dominant) antibody
    • Spastic paraplegia 17 (Silver syndrome) antibody
    • Spastic paraplegia 17 antibody
    • Spastic paraplegia with amyotrophy of hands and feet (Silver syndrome) antibody
    • Spastic paraplegia with amyotrophy of hands and feet antibody
    • SPG 17 antibody
    • SPG17 antibody
    see all

Anti-BSCL2 antibody 画像

  • Anti-BSCL2 antibody (ab107170) at 1/100 dilution + NCI-H460 cell line lysates at 35 µg

    Predicted band size : 44 kDa

Anti-BSCL2 antibody (ab107170) 使用論文

ab107170 has not yet been referenced specifically in any publications.

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