Anti-Saposin A antibody
An antibody raised against saposin A; a protein involved in immune system activation. Saposin A functions in sphingolipid catabolism in human lysosomes as well as loading of lipid antigens on to T cells.
Catalogue number crb2005643 Antibody Anti-Saposin A antibody Antigen Peptide KLH conjugated synthetic peptide crb1200852e Protein ID P07602 Human PSAP Aliases Prosaposin, Proactivator polypeptide, Saposin-A, Protein A, PSAP, GLBA, SAP1 Cross-Reactivity Human Target Protein Species Human Host Species Rabbit Antibody Type Polyclonal Concentration 1mg/ml Target Saposin A Storage Stabilisers The product should be stored at -20°C for short term storage and long term storage. Avoid repeated freeze/ thaw cycles. Family Hominidae Disease Area Combined saposin deficiency (CSAPD), Krabbe disease, atypical, due to saposin A deficiency (AKRD) Storage The product should be stored at -20°C for short term storage and long term storage. Avoid repeated freeze/ thaw cycles. References
Hulková et al., (2001). A novel mutation in the coding region of the prosaposin gene leads to a complete deficiency of prosaposin and saposins, and is associated with a complex sphingolipidosis dominated by lactosylceramide accumulation. Hum Mol Genet. 10(9): 927. PMID: 11309366.
Spiegel et al., (2005). A mutation in the saposin A coding region of the prosaposin gene in an infant presenting as Krabbe disease: first report of saposin A deficiency in humans. Mol Genet Metab. 84(2): 160. PMID: 15773042.
Saposins are specific low-molecular mass non-enzymic proteins, they participate in the lysosomal degradation of sphingolipids, which takes place by the sequential action of specific hydrolases. Saposins are also involved in trafficking and loading of lipids onto Cluster of Differentiation 1 (CD1) receptor proteins. These CD1 receptor proteins play a vital role in the innate and adaptive immune system by presenting these lipid antigens to T-cell receptors on the surface of T cells, triggering immune responses.
Saposin A activates the hydrolysis of galactosylceramide by b-galactosidase to produce ceramide and galactose; Saposin A deficiency causes galactosylceramide lipidosis. Combined saposin deficiency (CSAPD) is due to absence of all saposins, leading to a fatal storage disorder with hepatosplenomegaly and severe neurological involvement. Krabbe disease, atypical, due to saposin A deficiency (AKRD) is a disorder of galactosylceramide metabolism. Clinical features include infant neurologic regression hyporeflexia, generalized brain atrophy, and diffuse white matter demyelination.