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鞘脂激活蛋白原抗体

鞘脂激活蛋白原抗体

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产品名称： 鞘脂激活蛋白原抗体

英文名称： Parkin

产品编号： 1865

产品价格： null

产品产地： 上海

品牌商标： 雅吉

更新时间： null

使用范围： WB ELISA IHC-P IHC-F IF

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中文名称 鞘脂激活蛋白原抗体
别    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.  
研究领域 肿瘤  发育生物学  神经生物学  细胞周期蛋白  激酶和磷酸酶  脂蛋白  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse, Rat,  (predicted: Human, Dog, )
产品应用 WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 8.8/58kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Prosaposin:101-200/524 
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMed PubMed
产品介绍 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.

表达在正常的前列腺、前列腺增生和前列腺肿瘤中。
产品图片 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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