This gene encodes a serum protein found in association with the major histocompatibility complex (MHC) class I heavy chain on the surface of nearly all nucleated cells. The protein has a predominantly beta-pleated sheet structure that can form amyloid fibrils in some pathological conditions. A mutation in this gene has been shown to result in hypercatabolic hypoproteinemia.[provided by RefSeq, Sep 2009].
产品应用
应用
已检合格种属
预测种属
推荐稀释比例
WB
Human
1:500-2000
交叉反应
交叉反应: Human
相关产品
暂无相关产品
靶标
基因名
B2M
蛋白名
beta-2-microglobulin
亚基
Heterodimer of an alpha chain and a beta chain. Beta-2-microglobulin is the beta-chain of major histocompatibility complex class I molecules. Polymers of beta 2-microglobulin can be found in tissues from patients on long-term hemodialysis.
亚细胞定位
Secreted. Note=Detected in serum and urine.
翻译后修饰
Glycation of Ile-21 is observed in long-term hemodialysis patients.
疾病
Defects in B2M are the cause of hypercatabolic hypoproteinemia (HYCATHYP) [MIM:241600]. Affected individuals show marked reduction in serum concentrations of immunoglobulin and albumin, probably due to rapid degradation.
Note=Beta-2-microglobulin may adopt the fibrillar configuration of amyloid in certain pathologic states. The capacity to assemble into amyloid fibrils is concentration dependent. Persistently high beta(2)-microglobulin serum levels lead to amyloidosis in patients on long-term hemodialysis.
相似性
Belongs to the beta-2-microglobulin family.
Contains 1 Ig-like C1-type (immunoglobulin-like) domain.
功能
Component of the class I major histocompatibility complex (MHC). Involved in the presentation of peptide antigens to the immune system.