Rabbit anti-Human SAP Polyclonal Antibody | anti-SAP antibody

SAP, ID (E300) (Proactivator Polypeptide, Saposin-A, Protein A, Saposin-B-Val, Saposin-B, Sphingolipid Activator Protein 1, SAP-1, Cerebroside Sulfate Activator, CSAct, Dispersin, Sulfatide/GM1 Activator, Saposin-C, Co-beta-glucosidase, A1 Activator, Gluc

Cat. Num. AAA6500430

• Gene Names: PSAP; GLBA; SAP1
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A/G Affinity Chromatography.
• Synonyms: SAP; Polyclonal Antibody; ID (E300) (Proactivator Polypeptide; Saposin-A; Protein A; Saposin-B-Val; Saposin-B; Sphingolipid Activator Protein 1; SAP-1; Cerebroside Sulfate Activator; CSAct; Dispersin; Sulfatide/GM1 Activator; Saposin-C; Co-beta-glucosidase; A1 Activator; Gluc; anti-SAP antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human SAP.
• Purity/Purification: Purified by Protein A/G Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight405.

• Applicable Applications for anti-SAP antibody: FLISA, Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 285-314 in the central region of human SAP, UniProt Accession #NP_002769; P07602.
• Conjugate: MaxLight405
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight405 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot analysis in Y79, A2058, CEM cell line lysates (35ug/lane) usingMBS627000. SAP (arrow) was detected using the purified Pab.)

Related Product Information for anti-SAP antibody

MaxLight 405 is a new Violet photostable dye conjugate comparable to Alexa Fluor 405, PacificBlue, Brilliant Violet 421 and offers better labeling efficiency, brighter imaging and increased immunodetection. Absorbance (400nm); Emission (423nm); Extinction Coefficient 32,000.

Product Categories/Family for anti-SAP antibody

Antibodies; Proteins

References

1. Gunia, S., Virchows Arch. 454(5), 573-579 (2009). 2. Kuchar, L., Am. J. Med. Genet. A 149A(4), 613-621 (2009).

NCBI and Uniprot Product Information

• NCBI GI #: 11386147
• NCBI GeneID: 5660
• NCBI Accession #: NP_002769
• NCBI GenBank Nucleotide #: NM_002778.2
• UniProt Accession #: P07602; P07292; P15793; P78538; P78541; P78546; P78547; P78558; Q53Y86; Q6IBQ6; Q92739; Q92740
• Molecular Weight: 58,484 Da
• NCBI Official Full Name: prosaposin isoform a preproprotein
• NCBI Official Synonym Full Names: prosaposin
• NCBI Official Symbol: PSAP
• NCBI Official Synonym Symbols: GLBA; SAP1
• NCBI Protein Information: prosaposin
• UniProt Protein Name: Proactivator polypeptide
• Protein Family: Sapecin
• UniProt Gene Name: PSAP
• UniProt Synonym Gene Names: GLBA; SAP1; CSAct; SAP-1; SAP-2
• UniProt Entry Name: SAP_HUMAN

NCBI Description

This gene encodes a highly conserved glycoprotein which is a precursor for 4 cleavage products: 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, Tay-Sachs disease, and metachromatic leukodystrophy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]

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: 10q21-q22

Cellular Component: nucleoplasm; Golgi apparatus; extracellular space; lysosomal lumen; mitochondrion; intracellular membrane-bound organelle; lysosomal membrane; nucleolus; integral to membrane; extracellular region

Molecular Function: protein binding; enzyme activator activity; lipid binding

Biological Process: positive regulation of catalytic activity; platelet activation; regulation of lipid metabolic process; platelet degranulation; sphingolipid metabolic process; regulation of MAPKKK cascade; glycosphingolipid metabolic process; lipid transport; blood coagulation

Disease: Gaucher Disease, Atypical, Due To Saposin C Deficiency; Metachromatic Leukodystrophy Due To Saposin B Deficiency; Krabbe Disease, Atypical, Due To Saposin A Deficiency; Combined Saposin Deficiency