产品名称

POMT1抗体

英文名称

POMT1

产品规格

50ul  100ul 200ul 

产品货号

GOY-01K1449

产品分类

抗体 / 一抗

交叉反应

Human, Mouse, Rat, Pig, Cow, Horse

保存条件

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

标记

Unconjugated

抗体来源

Rabbit

抗体类型

Polyclonal

研究领域

肿瘤 细胞生物  神经生物学 糖蛋白

蛋白细胞定位

细胞浆

纯化方法

affinity purified by Protein A

性状

Liquid

浓度

1mg/ml

储存液

 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

发货地

上海

背景资料

POMT1 (Protein O mannosyl transferase 1) is a multipass membrane protein that is found in the endoplasmic reticulum. POMT1 catalyses the transfer of mannosyl residues to the hydroxyl groups of serine or threonine residues. Enzymatic activity is dependent on co expression of POMT1 with POMT2. Defects in the POMT1 e are associated with Walker-Warburg syndrome (WWS), a contial muscular dystrophy that is associated with mental retardation and is usually lethal within the first few months of life. Other defects in the POMT1 e result in limb girdle muscular dystrophy type 2K (LGMD2K), which is associated with mild mental retardation. Studies in Drosophila suggest that mutation of POMT1 alters the efficacy of synaptic transmission and a change in subunit composition of post synaptic glutamate receptors at the neuromuscular junction. Missense mutations in POMT1 have been associated with glioneuronal and glial brain tumours.