Overview

  • Product name
  • Description
    Rabbit polyclonal to PSAP
  • Tested applications
    Suitable for: ICC/IF, WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Orangutan
  • Immunogen

    Synthetic peptide conjugated to KLH derived from within residues 350 - 450 of Human PSAP.

  • Positive control
    • This antibody gave a positive signal in the following human tissue lysates: Kidney; Testis.

Properties

Applications

Our Abpromise guarantee covers the use of ab88251 in the following tested applications.

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

Application Abreviews Notes
ICC/IF Use a concentration of 5 µg/ml.
WB Use a concentration of 1 µg/ml. Detects a band of approximately 55 kDa (predicted molecular weight: 58 kDa).

Target

  • 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
  • Database links
  • Alternative names
    • 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

Images

  • All lanes : Anti-PSAP antibody (ab88251) at 1 µg/ml

    Lane 1 : Human kidney tissue lysate - total protein (ab30203)
    Lane 2 : Human testis tissue lysate - total protein (ab30257)

    Lysates/proteins at 10 µg per lane.

    Secondary
    Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 58 kDa
    Observed band size : 55 kDa (why is the actual band size different from the predicted?)


    Exposure time : 20 minutes
  • ICC/IF image of ab88251 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody ab88251 at 5µg/ml overnight at +4°C. The secondary antibody (green) was DyLight® 488 goat anti- rabbit IgG (H+L)(ab96899) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

References

ab88251 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab88251.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

Sign up