概述

  • 产品名称Anti-PSAP抗体
    参阅全部 PSAP 一抗
  • 描述
    兔多克隆抗体to PSAP
  • 经测试应用适用于: WB, IHC-Pmore details
  • 种属反应性
    与反应: Human
    预测可用于: Cow, Monkey
  • 免疫原

    Recombinant protein fragment corresponding to a region within amino acids 1 and 220 of Human PSAP (NP_002769)

  • 阳性对照
    • 293T, A431, HeLa and Molt-4 cell lines. Paraffin-embedded NCIN87 xenograft.

性能

应用

Our Abpromise guarantee covers the use of ab97441 in the following tested applications.

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

应用 Ab评论 说明
WB 1/500 - 1/3000. Predicted molecular weight: 58 kDa.
IHC-P 1/100 - 1/500.

靶标

  • 功能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).
  • 疾病相关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).
  • 序列相似性Contains 2 saposin A-type domains.
    Contains 4 saposin B-type domains.
  • 翻译后修饰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.
  • 细胞定位Lysosome.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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 图像

  • Anti-PSAP antibody (ab97441) at 1/2000 dilution + Molt-4 whole cell lysate at 30 µg

    Predicted band size : 58 kDa
  • Immunohistochemical analysis of paraffin-embedded NCIN87 xenograft using ab97441 at 1/500 dilution.

Anti-PSAP antibody (ab97441)参考文献

ab97441 has not yet been referenced specifically in any publications.

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