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PSAP抗体

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产品名称: PSAP抗体
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产品展商: XYbscience
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简单介绍

PSAP抗体该基因编码一个高度保守的蛋白,4的裂解产物的前体:A,B,C,和D每个域的前体蛋白约长80个氨基酸残基,与几乎相同的位置的半胱氨酸残基和糖基化位点。saposins A-D定位主要是溶酶体室在他们方便的短的寡糖组糖脂代谢。PSAP抗体前体蛋白既是分泌蛋白,又是一种整体膜蛋白,具有神经营养活性。在这个基因的突变已与高雪氏病、Tay-Sachs病,和异染性脑白质病变。选择性剪接导致编码不同亚型的多个转录变异体。[由RefSeq提供,月2008日]


PSAP抗体  的详细介绍

PSAP抗体特异性结合抗原:抗体本身不能直接溶解或杀伤带有特异抗原的靶细胞,通常需要补体或吞噬细胞等共同发挥效应以**病原微生物或导致病理损伤。然而,抗体可通过与病毒或**的特异性结合,直接发挥中和病毒的作用。

产品编号xy- 1879R

英文名称PSAP

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.  

说 明 书100ul  200ul

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

PSAP抗体交叉反应 Human, Mouse, Rat, Dog,

产品应用WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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Lyophilized or Liquid

浓    度1mg/1ml

免 疫 原KLH conjugated synthetic peptide derived from human Prosaposin

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.

PSAP抗体保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMedPubMed

产品介绍background:

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

PSAP抗体antibody, Ab)是由效应B细胞(效应**B细胞)分泌,机体用于抵御外来物质,如病毒,**等抗原,结构呈“Y”字型的球状蛋白质,仅仅存在于脊椎动物的血液和B**细胞膜表面。凡是能够跟抗体结合的物质,均被称作抗原,因此对于抗抗体(能够结合抗体的抗体)来说,抗体本身也是一种抗原物质。

   QQ图片20171030091318

PSAP抗体普通抗体重链和轻链的结构

重链结构:普通的**球蛋白具有2条重链(H链),分子量约为50kD,有μ、δ、γ、ε和α五种重链亚型,对应的**球蛋白名称分别为IgMIgGIgAIgDIgE

轻链结构:  普通**球蛋白具有2条轻链(L链),分子质量约25kDa,有κ链和λ链两种亚型,这两种轻链决定了Ig的亚型类别(IgG1IgG2IgG3IgG4)。一个天然的Ig分子两条轻链总是相同的,但在同一个体内可存在分别带有κ或λ链的抗体分子。不同种属生物体内两型轻链的比例不同,正常人血清**球蛋白κ链:λ链约为21,而在小鼠的比例为201

2.2抗体Fab段和Fc

IgG经木瓜蛋白酶酶切后裂解为2个完全相同的Fab段和1Fc,每个Fab段都为单价,可与抗原结合但不会再发生凝集反应;经胃蛋白酶酶切后裂解为1个完整F(ab)2片段和碎片化的Fc片段,F(ab)2片段为双价,可同时结合两个抗原表位。Fab段为抗原结合片段(fragment of antigen bindingFab),相当于抗体分子的两个臂,由一个完整的轻链和重链的VHCH1结构域组成。Fc段为可结晶段(fragment crystallizableFc)相当于IgCH2CH3结构域,是Ig与效应分子或者细胞相互作用的部位。Fab段包含完整的可变区,以及恒定区的CH1区域。Fc段仅指Ig恒定区CH2CH3的区域,相当于Y字结构下面那一部分。

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