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Products:Signal Transduction >> Metabolism >> Energy Metabolism
Overview
Product name
Anti-PSAP antibody
See all PSAP products (6) ...
Description
Rabbit polyclonal to PSAP
Tested applications
ELISA, ICC/IF, WB, IP, IHC-Frmore details
Cross reactivity
Reacts with
Mouse, Rat, Human
Immunogen
A synthetic peptide corresponding to an internal sequence of human PSAP.
Positive control
HepG2 whole cell lysate
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage buffer
Preservative: None
Constituents: Tris buffered saline, pH 7.2, containing antibody stabilizer
Concentration
Concentration information loading...
Purity
Immunogen affinity purified
Clonality
Polyclonal
Isotype
IgG
Research areas
Signal Transduction >> Metabolism >> Lipid metabolism
Signal Transduction >> Metabolism >> Energy Metabolism
Applications
Show applications key
Our Abpromise guarantee covers the use of ab68466 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
ELISA: Use a concentration of 0.01 - 0.1 µg/ml.
ICC/IF: Use a concentration of 1 µg/ml
WB: Use a concentration of 0.1 - 1 µg/ml.Detects a band of approximately 70 kDa (predicted molecular weight: 58 kDa).
IP: Use a concentration of 2 - 5 µg/ml.
IHC-Fr: Use at an assay dependent dilution. (See Abreview.)
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.
Target information above from: UniProt accessionP07602
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- A1 activator antibody
- Cerebroside sulfate activator antibody
- Co-beta-glucosidase antibody
see all
Database links
- Entrez Gene: 5660 Human
- Entrez Gene: 19156 Mouse
- Entrez Gene: 25524 Rat
- Omim: 176801 Human
- SwissProt: P07602 Human
- SwissProt: Q61207 Mouse
- SwissProt: P10960 Rat
- Unigene: 523004 Human
Anti-PSAP antibody images:
Western blot - PSAP antibody (ab68466)
Anti-PSAP antibody (ab68466) at 1/500 dilution + HepG2 whole cell lysate
Predicted band size : 58 kDa
Observed band size : 70 kDa (why is the actual band size different from the predicted?)
Immunocytochemistry/ Immunofluorescence-PSAP antibody(ab68466)
ICC/IF image of ab68466 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 (ab68466, 1µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) 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 (ab68466)
This product has been referenced in:
- Jain MRet al. Altered proteolytic events in experimental autoimmune encephalomyelitis discovered by iTRAQ shotgun proteomics analysis of spinal cord. Proteome Sci 7:25 (2009). WB; Rat.Read more (PubMed: 19607715) »
See 1 publication for this product
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"