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Products:Signal Transduction >> Metabolism >> Energy Metabolism
Overview
Product name
Anti-PSAP antibody
See all PSAP products (6) ...
Description
Mouse monoclonal to PSAP
Tested applications
WB, IHC-Pmore details
Cross reactivity
Reacts with
Human
Immunogen
Recombinant full length protein, corresponding to amino acids 18-525 of Human PSAP
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
PBS, pH 7.2
Concentration
Concentration information loading...
Purity
Protein G purified
Clonality
Monoclonal
Isotype
IgG2a
Light chain type
kappa
Research areas
Signal Transduction >> Metabolism >> Lipid metabolism
Signal Transduction >> Metabolism >> Energy Metabolism
Applications
Show applications key
Our Abpromise guarantee covers the use of ab55624 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application notes
WB: Use at a concentration of 1-5 µg/ml.
IHC-P: Use at a concentration of 5 µg/ml.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
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
- Omim: 176801 Human
- SwissProt: P07602 Human
- Unigene: 523004 Human
Anti-PSAP antibody images:
IHC-P - PSAP antibody (ab55624)
PSAP antibody (ab55624) used in immunohistochemistry at 5ug/ml on formalin fixed and paraffin embedded human spleen tissue.
Western blot - PSAP antibody (ab55624)
Predicted band size : 58 kDa
Observed band size : 58 kDa
Additional bands at : 48 kDa. We are unsure as to the identity of these extra bands.
Western Blot analysis of PSAP expression in transfected 293T cell line by PSAP monoclonal antibody.
Lane 1: PSAP transfected lysate.
Lane 2: Non-transfected lysate.
References for Anti-PSAP antibody (ab55624)
ab55624 has not yet been referenced specifically in any publications.
Publishing research using ab55624? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"