製品の概要

製品の詳細

  • 由来
    Recombinant
  • 由来
    Escherichia coli
  • アミノ酸配列
    • 生物種
      Human
    • 配列
      MMMHMDQQPKEICALVGFCDEVKEMPMQTLVPAKVASKNVIPALELVEPI KKH EVPAKSDVYCEVCEFLVKEVTKLIDNNKTEKEILDAFDKMCSKLP KSLSEECQ EVVDTYGSSILSILLEEVSPELVCSMLHLCSGTRLPALTV HVTQPKDGGFCEV CKKLVGYLDRNLEKNSTKQEILAALEKGCSFLPDP YQKQCDQFVAEYEPVLIE ILVEVMDPSFVCLKIGACPSAHKPLLGTEK CIWGPSYWCQNTETAAQCNAVEH CKRHVWN
    • 領域
      255 to 526

特性

Our Abpromise guarantee covers the use of ab93982 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

    SDS-PAGE

  • 製品の状態
    Lyophilised
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped at 4°C. Store at 4°C prior to reconstitution. Store at -80°C.

    Preservative: None
    Constituents: 0.5% Trehalose, 6M Urea, 100mM Sodium phosphate, 10mM Sodium chloride, pH 4.5

  • 再構成
    Reconstitute with 58 µl aqua dest.

関連情報

  • 別名
    • A1 activator
    • Cerebroside sulfate activator
    • Co-beta-glucosidase
    • Component C
    • CSAct
    • Dispersin
    • GLBA
    • Glucosylceramidase activator
    • Proactivator polypeptide
    • Proactivator polypeptide precursor
    • Prosaposin
    • Prosaposin (sphingolipid activator protein 1)
    • prosaposin (variant Gaucher disease and variant metachromatic leukodystrophy)
    • Protein A
    • Protein C
    • PSAP
    • SAP-1
    • SAP-2
    • SAP_HUMAN
    • SAP1
    • Saposin A
    • Saposin B
    • Saposin B Val
    • Saposin C
    • Saposin D
    • Saposin-D
    • Saposins
    • Sgp1
    • Sphingolipid activator protein 1
    • Sphingolipid activator protein 2
    • Sulfated glycoprotein 1
    • Sulfatide/GM1 activator
    see all
  • 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

画像

  • The image shows an electrophoretic assay performed using an Agilent 5100 ALP. In some images coloured control bands can be seen at 15 kDa (green) and/or 240 kDa (purple). The protein-specific band is blue.

参考文献

ab93982 has not yet been referenced specifically in any publications.

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