protein

Human Beta-2-microglobulin protein

MBS717426

1 mg

735 USD

Native Protein

Human Beta-2-microglobulin protein

Beta-2-microglobulin

Full length native Beta-2-microglobulin protein

beta-2-microglobulin; Beta-2-microglobulin; beta-2-microglobulin; beta-2-microglobin; beta chain of MHC class I molecules; beta-2-microglobulin

B2M; B2M

B2MG_HUMAN

Purified from human urine

>95% (SDS-PAGE)

Liquid

Aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze / thaw cycles.

SDS-PAGE, ELISA (EIA), Western Blot (WB)

Peptide: Full length native Beta-2-microglobulin protein

Storage Buffer: PBS

Component of the class I major histocompatibility complex (MHC). Involved in the presentation of peptide antigens to the immune system. Defects in B2M are the cause of hypercatabolic hypoproteinemia (HYCATHYP) . Affected individuals show marked reduction in serum concentrations of immunoglobulin and albumin, probably due to rapid degradation.

179318

AAA51811.1

P61769

13,715 Da

Adaptive Immune System Pathway (366160); Amyloids Pathway (366238); Antigen Presentation: Folding, Assembly And Peptide Loading Of Class I MHC Pathway (366163); Antigen Processing And Presentation Pathway (83074); Antigen Processing And Presentation Pathway (485); Antigen Processing-Cross Presentation Pathway (477122); Class I MHC Mediated Antigen Processing Presentation Pathway (366161); Cytokine Signaling In Immune System Pathway (366171); Disease Pathway (530764); Downstream Signaling In Naive CD8+ T Cells Pathway (138018)

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]

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.

1 mg

735 USD