Anti-Protective protein/Cathepsin A (PPCA) 抗体 [MM0125-14H2] (ab89308)

製品の概要

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab89308 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 1/500 - 1/1000. Predicted molecular weight: 54 kDa.

ターゲット情報

  • 機能Protective protein appears to be essential for both the activity of beta-galactosidase and neuraminidase, it associates with these enzymes and exerts a protective function necessary for their stability and activity. This protein is also a carboxypeptidase and can deamidate tachykinins.
  • 関連疾患Defects in CTSA are the cause of galactosialidosis (GSL) [MIM:256540]. A lysosomal storage disease associated with a combined deficiency of beta-galactosidase and neuraminidase, secondary to a defect in cathepsin A. All patients have clinical manifestations typical of a lysosomal disorder, such as coarse facies, cherry red spots, vertebral changes, foam cells in the bone marrow, and vacuolated lymphocytes. Three phenotypic subtypes are recognized. The early infantile form is associated with fetal hydrops, edema, ascites, visceromegaly, skeletal dysplasia, and early death. The late infantile type is characterized by hepatosplenomegaly, growth retardation, cardiac involvement, and a normal or mildly affected mental state. The juvenile/adult form is characterized by myoclonus, ataxia, angiokeratoma, mental retardation, neurologic deterioration, absence of visceromegaly, and long survival.
  • 配列類似性Belongs to the peptidase S10 family.
  • 細胞内局在Lysosome.
  • Information by UniProt
  • 参照データベース
  • 別名
    • beta galactosidase 2 antibody
    • BETA GALACTOSIDASE PROTECTIVE PROTEIN antibody
    • beta-galactosidase 2 antibody
    • beta-galactosidase protective protein antibody
    • betagalactosidase 2 antibody
    • Carboxypeptidase C antibody
    • Carboxypeptidase L antibody
    • carboxypeptidase Y-like kininase antibody
    • Cathepsin A antibody
    • Ctsa antibody
    • deamidase antibody
    • EC 3.4.16.5 antibody
    • Glactosialidosis antibody
    • GLB2 antibody
    • Goldberg Syndrome antibody
    • GSL antibody
    • lysosomal carboxypeptidase A antibody
    • Lysosomal protective protein 20 kDa chain antibody
    • Lysosomal protective protein antibody
    • Lysosomal protective protein deficiency antibody
    • NEURAMINIDASE BETA GALACTOSIDASE EXPRESSION; NGBE antibody
    • Neuraminidase deficiency with beta-galactosidase deficiency antibody
    • NGBE antibody
    • OTTHUMP00000031778 antibody
    • OTTHUMP00000031781 antibody
    • PPCA antibody
    • PPCA deficiency antibody
    • PPGB antibody
    • PPGB_HUMAN antibody
    • Protective protein cathepsin A antibody
    • Protective protein for beta galactosidase antibody
    • Protective protein for beta-galactosidase antibody
    • Protective protein/cathepsin A deficiency antibody
    • urinary kininase antibody
    see all

Anti-Protective protein/Cathepsin A (PPCA) antibody [MM0125-14H2] 画像

  • Anti-Protective protein/Cathepsin A (PPCA) antibody [MM0125-14H2] (ab89308) at 1/1000 dilution + Human liver tissue

    Predicted band size : 54 kDa

Anti-Protective protein/Cathepsin A (PPCA) antibody [MM0125-14H2] (ab89308) 使用論文

ab89308 has not yet been referenced specifically in any publications.

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