製品の概要

  • 製品名Anti-PSAP antibody
    PSAP 一次抗体 製品一覧
  • 製品の詳細
    Mouse monoclonal to PSAP
  • アプリケーション適用あり: WB, IHC-Pmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Recombinant full length protein, corresponding to amino acids 18-525 of Human PSAP

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab55624 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
IHC-P
  • 追加情報WB: Use at a concentration of 1-5 µg/ml.
    IHC-P: Use at a concentration of 5 µg/ml.


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • ターゲット情報

    • 機能The lysosomal degradation of sphingolipids takes place by the sequential action of specific hydrolases. Some of these enzymes require specific low-molecular mass, non-enzymic proteins: the sphingolipids activator proteins (coproteins).
      Saposin-A and saposin-C stimulate the hydrolysis of glucosylceramide by beta-glucosylceramidase (EC 3.2.1.45) and galactosylceramide by beta-galactosylceramidase (EC 3.2.1.46). Saposin-C apparently acts by combining with the enzyme and acidic lipid to form an activated complex, rather than by solubilizing the substrate.
      Saposin-B stimulates the hydrolysis of galacto-cerebroside sulfate by arylsulfatase A (EC 3.1.6.8), GM1 gangliosides by beta-galactosidase (EC 3.2.1.23) and globotriaosylceramide by alpha-galactosidase A (EC 3.2.1.22). Saposin-B forms a solubilizing complex with the substrates of the sphingolipid hydrolases.
      Saposin-D is a specific sphingomyelin phosphodiesterase activator (EC 3.1.4.12).
    • 関連疾患Defects in PSAP are the cause of combined saposin deficiency (CSAPD) [MIM:611721]; also known as prosaposin deficiency. CSAPD is due to absence of all saposins, leading to a fatal storage disorder with hepatosplenomegaly and severe neurological involvement.
      Defects in PSAP saposin-B region are the cause of leukodystrophy metachromatic due to saposin-B deficiency (MLD-SAPB) [MIM:249900]. MLD-SAPB is an atypical form of metachromatic leukodystrophy. It is characterized by tissue accumulation of cerebroside-3-sulfate, demyelination, periventricular white matter abnormalities, peripheral neuropathy. Additional neurological features include dysarthria, ataxic gait, psychomotr regression, seizures, cognitive decline and spastic quadriparesis.
      Defects in PSAP saposin-C region are the cause of atypical Gaucher disease (AGD) [MIM:610539]. Affected individuals have marked glucosylceramide accumulation in the spleen without having a deficiency of glucosylceramide-beta glucosidase characteristic of classic Gaucher disease, a lysosomal storage disorder.
      Defects in PSAP saposin-A region are the cause of atypical Krabbe disease (AKRD) [MIM:611722]. AKRD is a disorder of galactosylceramide metabolism. AKRD features include progressive encephalopathy and abnormal myelination in the cerebral white matter resembling Krabbe disease.
      Note=Defects in PSAP saposin-D region are found in a variant of Tay-Sachs disease (GM2-gangliosidosis).
    • 配列類似性Contains 2 saposin A-type domains.
      Contains 4 saposin B-type domains.
    • 翻訳後修飾This precursor is proteolytically processed to 4 small peptides, which are similar to each other and are sphingolipid hydrolase activator proteins.
      N-linked glycans show a high degree of microheterogeneity.
      The one residue extended Saposin-B-Val is only found in 5% of the chains.
    • 細胞内局在Lysosome.
    • Information by UniProt
    • 参照データベース
    • 別名
      • A1 activator antibody
      • Cerebroside sulfate activator antibody
      • Co-beta-glucosidase antibody
      • Component C antibody
      • CSAct antibody
      • Dispersin antibody
      • GLBA antibody
      • Glucosylceramidase activator antibody
      • Proactivator polypeptide antibody
      • Proactivator polypeptide precursor antibody
      • Prosaposin (sphingolipid activator protein 1) antibody
      • prosaposin (variant Gaucher disease and variant metachromatic leukodystrophy) antibody
      • Prosaposin antibody
      • Protein A antibody
      • Protein C antibody
      • PSAP antibody
      • SAP-1 antibody
      • SAP-2 antibody
      • SAP_HUMAN antibody
      • SAP1 antibody
      • Saposin A antibody
      • Saposin B antibody
      • Saposin B Val antibody
      • Saposin C antibody
      • Saposin D antibody
      • Saposin-D antibody
      • Saposins antibody
      • Sgp1 antibody
      • Sphingolipid activator protein 1 antibody
      • Sphingolipid activator protein 2 antibody
      • Sulfated glycoprotein 1 antibody
      • Sulfatide/GM1 activator antibody
      see all

    Anti-PSAP antibody 画像

    • PSAP antibody (ab55624) used in immunohistochemistry at 5ug/ml on formalin fixed and paraffin embedded human spleen tissue.


    • Predicted band size : 58 kDa
      Observed band size : 58 kDa
      Additional bands at : 48 kDa. We are unsure as to the identity of these extra bands.

    Anti-PSAP antibody (ab55624) 使用論文

    ab55624 has not yet been referenced specifically in any publications.

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