Beta 2-Microglobulin (B2M) Antibody – Mouse Monoclonal

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.

Function: Component of the class I major histocompatibility complex (MHC). Involved in the presentation of peptide antigens to the immune system.

Subunit structure: 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. Ref.14

Subcellular location: Secreted. Note: Detected in serum and urine. Ref.16 Ref.17

Post-translational modification: Glycation of Ile-21 is observed in long-term hemodialysis patients.

Involvement in disease: 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. Ref.33Note=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. Ref.13 Ref.14 Ref.15 Ref.16 Ref.17 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31 Ref.32

Sequence similarities: Belongs to the beta-2-microglobulin family.Contains 1 Ig-like C1-type (immunoglobulin-like) domain.