Overview

Description

  • Nature
    Synthetic

Associated products

Specifications

Our Abpromise guarantee covers the use of ab106466 in the following tested applications.

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

  • Purity
    70 - 90% by HPLC.

  • Additional notes

    - First try to dissolve a small amount of peptide in either water or buffer. The more charged residues on a peptide, the more soluble it is in aqueous solutions.
    - If the peptide doesn’t dissolve try an organic solvent e.g. DMSO, then dilute using water or buffer.
    - Consider that any solvent used must be compatible with your assay. If a peptide does not dissolve and you need to recover it, lyophilise to remove the solvent.
    - Gentle warming and sonication can effectively aid peptide solubilisation. If the solution is cloudy or has gelled the peptide may be in suspension rather than solubilised.
    - Peptides containing cysteine are easily oxidised, so should be prepared in solution just prior to use.

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    Information available upon request.

General Info

  • Alternative names
    • 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
  • Function
    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).
  • Involvement in disease
    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).
  • Sequence similarities
    Contains 2 saposin A-type domains.
    Contains 4 saposin B-type domains.
  • Post-translational
    modifications
    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.
  • Cellular localization
    Lysosome.
  • Information by UniProt

References

ab106466 has not yet been referenced specifically in any publications.

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