Anti-PSAP antibody (ab88251)
- Product nameAnti-PSAP antibodySee all PSAP primary antibodies ...
- DescriptionRabbit polyclonal to PSAP
- Tested applicationsICC/IF, WB more details
- Species reactivityReacts with: Human
Predicted to work with: Orangutan
Synthetic peptide conjugated to KLH derived from within residues 350 - 450 of Human PSAP.
- Positive controlThis antibody gave a positive signal in the following human tissue lysates: Kidney; Testis.
- Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
- Storage bufferPreservative: 0.02% Sodium Azide
Constituents: 1% BSA, PBS, pH 7.4
- Concentration information loading...
- PurityImmunogen affinity purified
- Clonality Polyclonal
- Research Areas
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.
|ICC/IF||ICC/IF: Use a concentration of 5 µg/ml.|
|WB||WB: Use a concentration of 1 µg/ml. Detects a band of approximately 55 kDa (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 220.127.116.11) and galactosylceramide by beta-galactosylceramidase (EC 18.104.22.168). 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 22.214.171.124), GM1 gangliosides by beta-galactosidase (EC 126.96.36.199) and globotriaosylceramide by alpha-galactosidase A (EC 188.8.131.52). Saposin-B forms a solubilizing complex with the substrates of the sphingolipid hydrolases.
Saposin-D is a specific sphingomyelin phosphodiesterase activator (EC 184.108.40.206).
- 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.
- Cellular localizationLysosome.
- A1 activator antibodyCerebroside sulfate activator antibodyCo-beta-glucosidase antibody
- Component C antibodyCSAct antibodyDispersin antibodyGLBA antibodyGlucosylceramidase activator antibodyProactivator polypeptide antibodyProactivator polypeptide precursor antibodyProsaposin (sphingolipid activator protein 1) antibodyprosaposin (variant Gaucher disease and variant metachromatic leukodystrophy) antibodyProsaposin antibodyProtein A antibodyProtein C antibodyPSAP antibodySAP-1 antibodySAP-2 antibodySAP_HUMAN antibodySAP1 antibodySaposin A antibodySaposin B antibodySaposin B Val antibodySaposin C antibodySaposin D antibodySaposin-D antibodySaposins antibodySgp1 antibodySphingolipid activator protein 1 antibodySphingolipid activator protein 2 antibodySulfated glycoprotein 1 antibodySulfatide/GM1 activator antibody
Anti-PSAP antibody images
All lanes : Anti-PSAP antibody (ab88251) at 1 µg/ml
Lane 1 :
Kidney (Human) Tissue Lysate - adult normal tissue (ab30203)
Lane 2 :
Testis (Human) Tissue Lysate - adult normal tissue (ab30257)
Lysates/proteins at 10 µg per lane.
Goat polyclonal Secondary Antibody to Rabbit IgG - H&L (HRP), pre-adsorbed (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 for Anti-PSAP antibody (ab88251)
ab88251 has not yet been referenced specifically in any publications.