Product nameAnti-PSAP antibody
See all PSAP primary antibodies
DescriptionRabbit polyclonal to PSAP
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Human
Synthetic peptide conjugated to KLH, corresponding to a region within internal amino acids 293-322 of Human PSAP.
- Y79, A2058 and CEM cell line lysates.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term.
Storage bufferPreservative: 0.09% Sodium azide
Constituent: 99% PBS
Concentration information loading...
PurityAmmonium Sulphate Precipitation
Purification notesab135654 is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
Our Abpromise guarantee covers the use of ab135654 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/50 - 1/100. Predicted molecular weight: 58 kDa.|
FunctionThe 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 184.108.40.206) and galactosylceramide by beta-galactosylceramidase (EC 220.127.116.11). 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 18.104.22.168), GM1 gangliosides by beta-galactosidase (EC 22.214.171.124) and globotriaosylceramide by alpha-galactosidase A (EC 126.96.36.199). Saposin-B forms a solubilizing complex with the substrates of the sphingolipid hydrolases.
Saposin-D is a specific sphingomyelin phosphodiesterase activator (EC 188.8.131.52).
Involvement in diseaseDefects 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 similaritiesContains 2 saposin A-type domains.
Contains 4 saposin B-type domains.
modificationsThis 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.
- Information by UniProt
- A1 activator antibody
- Cerebroside sulfate activator antibody
- Co-beta-glucosidase antibody
ab135654 has not yet been referenced specifically in any publications.