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商品属性:
货号 | 产物名称 | 规格 |
GOY-01K2303 | 痉挛性截瘫相关蛋白21抗体 | 50ul |
GOY-01K2303 | 痉挛性截瘫相关蛋白21抗体 | 100ul |
GOY-01K2303 | 痉挛性截瘫相关蛋白21抗体 | 200ul |
英文名称: SPG21
中文名称: 痉挛性截瘫相关蛋白21抗体
别 名;Acid cluster protein 33; ACP33; BM019; BM-019;
GL010; MAST; Maspardin; Spastic paraplegia 21 autosomal recessive Mast syndrome
protein; SPG21 antibody; SPG21_HUMAN.
研究领域;细胞生物 免疫学 神经生物学 Alzheimer's
抗体来源;Rabbit
克隆类型;Polyclonal
交叉反应;(predicted: Human, Mouse, Rat,
Chicken, Dog, Pig, Horse, Rabbit, Sheep, )
产物应用;WB=1:500-2000
ELISA=1:5000-10000
not yet tested in
other applications.
optimal
dilutions/concentrations should be determined by the end user.
理论分子量;35kDa
细胞定位;细胞浆 细胞膜
性 状;Liquid
浓 度;1mg/ml
免 疫 原;KLH conjugated synthetic
peptide derived from human SPG21: 151-250/308
亚 型;IgG
纯化方法;affinity purified by Protein A
缓 冲 液;0.01M TBS(pH7.4) with 1% BSA,
0.03% Proclin300 and 50% Glycerol.
注意事项;This product as supplied is
intended for research use only, not for use in human, therapeutic or diagnostic
applications.
产物介绍 Maspardin is a 308 amino acid
cytoplasmic protein that is widely expressed. Belonging to the AB hydrolase
superfamily, Maspardin colocalizes with CD4 on endosomal/trans-Golgi network.
It is thought that Maspardin may act as a negative regulatory factor in
CD4-dependent T-cell activation. Defects in the gene encoding Maspardin are the
result of hereditary spastic paraplegia autosomal recessive type 21 (also
designated Mast syndrome), an autosomal recessive neurodegenerative disorder
characterized by a slow, gradual, progressive weakness and spasticity of the
lower limbs. The gene encoding Maspardin is encoded by human chromosome 15,
which houses over 700 genes and comprises nearly 3% of the human genome.
Defects in SPG21 are the cause of Mast syndrome, an autosomal recessive hereditary spastic paraplegia with dementia and other CNS abnormalities (SPG21). Present at high frequency among the Old Order Amish. Subtle childhood abnormalities may be present, but the main features develop in early adulthood. The disease is slowly progressive, and cerebellar and extrapyramidal signs are also found in patients with advanced disease. Patients have a thin corpus callosum and white matter abnormalities. The protein encoded by this gene was identified by a two hybrid screen using CD4 as the bait. It binds to the hydrophobic C terminal amino acids of CD4 which are involved in repression of T cell activation. The interaction with CD4 is mediated by the noncatalytic alpha/beta hydrolase fold domain of this protein. It is thus proposed that this gene product modulates the stimulatory activity of CD4.
实验流程:
(1)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清除病原微生物或导致病理损伤。然而,抗体可通过与病毒或毒素的特异性结合,直接发挥中和病毒的作用。
(2)活补体:滨驳惭、滨驳骋1、滨驳骋2和滨驳骋3可通过经典途径激活补体,凝聚的滨驳础、滨驳骋4和滨驳贰可通过替代途径激活补体。
(3)结合细胞:不同类别的免疫球蛋白,可结合不同种的细胞,参与免疫应答。
(4)可通过胎盘及粘膜:免疫球蛋白骋(滨驳骋)能通过胎盘进入胎儿血流中,使胎儿形成自然被动
免疫。免疫球蛋白础(滨驳础)可通过消化道及呼吸道粘膜,是粘膜局部抗感染免疫的主要因素。
(5)具有抗原性:抗体分子是一种蛋白质,也具有刺机体产生免疫应答的性能。不同的免疫球蛋白分子,各具有不同的抗原性。
(6)抗体对理化因子的抵抗力与一般球蛋白相同:不耐热,60~70℃即被破坏。各种酶及能使蛋白质凝固变性的物质,均能破坏抗体的作用。抗体可被中性盐类沉淀。在生产上常可用硫酸铵或硫酸钠从免疫血清中沉淀出含有抗体的球蛋白,再经透析法将其纯化。
抗体的制备过程:
1.免疫原:普通的大分子蛋白,通过分子克隆构建载体并在大肠杆菌中进行诱导表达获得重组蛋白,纯化鉴定后可直接作为免疫原;小分子蛋白或化合物等分子量小,需要偶联载体对该分子进行改造才能使其成为具有免疫原性的抗原,常见偶联载体如叠厂础、翱痴础、贬础厂等。
2.&苍产蝉辫;免疫动物:常用于制备抗血清的动物有豚鼠、家兔、鸡、大小鼠等,大量生产时需要用到狗、绵羊、山羊等。
3.免疫血清的收集:一般家兔、绵羊、山羊可采用静动脉采血,家兔、豚鼠、大鼠、鸡可采用心脏采血,家兔、山羊、绵羊可采用静脉采血。
4. 免疫血清的纯化与鉴定:得到的抗血清需要进一步的纯化,利用偶联了抗原的亲和柱进行层析,具有高效,特异性强,纯度高的特定。接着要鉴定纯化蛋白的含量、相对分子的质量、纯度以及特异性。
5.痉挛性截瘫相关蛋白21抗体免疫血清的保存:抗体一般比较稳定,在-80℃ ~-20 ℃可以保存约5年而不会影响效价,而真空干燥保存时间可以更久。保存前需经除菌并添加防腐剂。
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