Knockout Validation
(Knockout Validation: Lane 1: Wild-type 293T cell lysate; Lane 2: PSAP knockout 293T cell lysate; Predicted MW: 58kd Observed MW: 60kd Primary Ab: 2ug/ml Rabbit Anti-Human PSAP Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (#MBS2086047))
Rabbit anti-Human Prosaposin (PSAP) Polyclonal Antibody | anti-PSAP antibody
FITC-Linked Polyclonal Antibody to Prosaposin (PSAP)
Knockout Validation
(Knockout Validation: Lane 1: Wild-type 293T cell lysate; Lane 2: PSAP knockout 293T cell lysate; Predicted MW: 58kd Observed MW: 60kd Primary Ab: 2ug/ml Rabbit Anti-Human PSAP Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (#MBS2086047))
Knockout Validation
(Knockout Validation: Lane 1: Wild-type 293T cell lysate; Lane 2: PSAP knockout 293T cell lysate; Predicted MW: 58kd Observed MW: 60kd Primary Ab: 2ug/ml Rabbit Anti-Human PSAP Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (#MBS2086047))
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a highly conserved preproprotein that is proteolytically processed to generate four main cleavage products including saposins A, B, C, and D. Each domain of the precursor protein is approximately 80 amino acid residues long with nearly identical placement of cysteine residues and glycosylation sites. Saposins A-D localize primarily to the lysosomal compartment where they facilitate the catabolism of glycosphingolipids with short oligosaccharide groups. The precursor protein exists both as a secretory protein and as an integral membrane protein and has neurotrophic activities. Mutations in this gene have been associated with Gaucher disease and metachromatic leukodystrophy. Alternative splicing results in multiple transcript variants, at least one of which encodes an isoform that is proteolytically processed. [provided by RefSeq, Feb 2016]
Uniprot Description
PSAP: 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). Defects in PSAP are the cause of combined saposin deficiency (CSAPD); 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). 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). 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). AKRD is a disorder of galactosylceramide metabolism. AKRD features include progressive encephalopathy and abnormal myelination in the cerebral white matter resembling Krabbe disease. Defects in PSAP saposin-D region are found in a variant of Tay-Sachs disease (GM2-gangliosidosis). 3 isoforms of the human protein are produced by alternative splicing.
Chromosomal Location of Human Ortholog: 10q22.1
Cellular Component: cytoplasm; extracellular region; extracellular space; integral to membrane; lysosomal lumen; lysosomal membrane; lysosome
Molecular Function: enzyme activator activity; G-protein-coupled receptor binding; lipid binding; protein binding
Biological Process: G-protein signaling, adenylate cyclase inhibiting pathway; glycosphingolipid metabolic process; lipid transport; platelet degranulation; regulation of autophagy; regulation of lipid metabolic process
Disease: Combined Saposin Deficiency; Gaucher Disease, Atypical, Due To Saposin C Deficiency; Krabbe Disease, Atypical, Due To Saposin A Deficiency; Metachromatic Leukodystrophy Due To Saposin B Deficiency
Research Articles on PSAP
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Product Notes
The PSAP psap (Catalog #AAA2068019) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The FITC-Linked Polyclonal Antibody to Prosaposin (PSAP) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Prosaposin (PSAP) can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (ELISA). Researchers should empirically determine the suitability of the PSAP psap for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Prosaposin (PSAP), Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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