| 中文名稱 | 鞘脂激活蛋白原抗體 |
| 別 名 | Prosaposin; A1 activator; Cerebroside sulfate activator; Co-beta-glucosidase; Component C; CSAct; Dispersin; GLBA; Glucosylceramidase activator; Proactivator polypeptide; Proactivator polypeptide precursor; Prosaposin (sphingolipid activator protein 1); prosaposin (variant Gaucher disease and variant metachromatic leukodystrophy); Protein A; Protein C; PSAP; SAP-1; SAP-2; SAP_HUMAN; SAP1; Saposin A; Saposin B; Saposin B Val; Saposin C; Saposin D; Saposin-D; Saposins; Sgp1; Sphingolipid activator protein 1; Sphingolipid activator protein 2; Sulfated glycoprotein 1; Sulfatide/GM1 activator. |
| 研究領域 | 腫瘤 發育生物學 神經生物學 細胞周期蛋白 激酶和磷酸酶 脂蛋白 新陳代謝 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應 | Mouse, Rat, (predicted: Human, Dog, ) |
| 產品應用 | WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 8.8/58kDa |
| 細胞定位 | 細胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Prosaposin:101-200/524 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 產品介紹 | 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] 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). Subunit: Saposin-B is a homodimer. Subcellular Location: Lysosome. 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. 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). Similarity: Contains 2 saposin A-type domains. Contains 4 saposin B-type domains. SWISS: P07602 Gene ID: 5660 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 Unigene: 277498 Mouse Unigene: 97173 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 表達在正常的前列腺、前列腺增生和前列腺腫瘤中。 |
| 產品圖片 |  Protein:testis(rat) lysates at 30ug; brain(rat) lysates at 30ug; Primary: Anti-Anti-PSAP/PAP (bs-1879R) at 1:200; Secondary: HRP conjugated Goat Anti-Rabbit IgG(bs-0295G-HRP) at 1: 3000; ECL excitated the fluorescence; Predicted band size : 58kD Observed band size : 58kD  Sample:Testis (Rat) Lysate at 40 ug BV-2 (Mouse) CellLysate at 30 ug Primary: Anti-PSAP (bs-1879R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 8.8/58 kD Observed band size: 58 kD  Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (PSAP) Polyclonal Antibody, Unconjugated (bs-1879R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining. Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-PSAP/PAP Polyclonal Antibody, Unconjugated(bs-1879R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining |
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