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。
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IgM、IgG、IgA、IgD和IgE。
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（IgG1，IgG2，IgG3，IgG4）。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2：1，而在小鼠的比例为20：1。
2.2抗体Fab段和Fc段
IgG经木瓜蛋白酶酶切后裂解为2个完全相同的Fab段和1个Fc段,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 binding，Fab），相当于抗体分子的两个臂，由一个完整的轻链和重链的VH和CH1结构域组成。Fc段为可结晶段（fragment crystallizable，Fc）相当于Ig的CH2和CH3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CH2和CH3的区域，相当于Y字结构下面那一部分。
合格 合格 Histone H3 (tri methyl K36) **基化组蛋白H3抗体
合格 Histone H3 (Di Methyl K36) 二甲基化组蛋白H3抗体
合格 合格 合格 Histone H3 (Di Methyl K4) 二甲基化组蛋白H3K4抗体
合格 Histone H3 (Di Methyl K9) 二甲基化组蛋白H3K9抗体
合格 Histone H3 (Tri Methyl K27) **基化组蛋白H3抗体
合格 合格 Histone H3(Acetyl K23) 乙酰化组蛋白H3抗体
合格 Histone H3 (Acetyl K18) 乙酰化组蛋白H3抗体
合格 合格 合格 Histone H3 (acetyl K9) 乙酰化组蛋白H3抗体
合格 ASF1A 细胞衰老相关蛋白ASF1A抗体
合格 SCD1 固醇酰辅酶A脱氢酶1抗体
合格 PDK1/Mitochondrial Pyruvate dehydrogenase kinase 1 丙酮酸脱氢酶激酶1抗体
合格 Perilipin A 脂滴包被蛋白Perilipin-A抗体
合格 CD21 2型补体受体抗体
合格 MSH6 错配修复蛋白6抗体
合格 合格 PGP9.5 神经细胞胞浆蛋白9.5抗体（蛋白基因产物9.5）
合格 合格 Oct-4B(OCT4B-190)NT 胚胎干细胞关键蛋白190抗体(N端)
合格 Piwil2 piwi样2蛋白抗体
合格 GDF15 生长分化因子15/巨嗜细胞抑制因子1抗体
合格 BAP135/TFII I 蛋白酪氨酸激酶BAP135抗体
合格 Cyclin G2 周期素G2抗体
合格 MeCP2 甲基化CpG结合蛋白2抗体