Human beta two microglobulin: Difference between revisions
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== | =Human Beta two microglobulin (b2m)= | ||
==Beta two microglubulin in human class I major histocompatibility complex== | |||
Human β2-Microglobulin (b2m) is the non-covalently bound light chain of the human class I major histocompatibility complex | |||
(MHC-I). its function is to ensure proper folding and cell-surface expression of MHC-1. | (MHC-I). its function is to ensure proper folding and cell-surface expression of MHC-1. | ||
The natural turnover of MHC-I gives rise to the release of b2m into plasmatic fluids at ~0.1 um and to its catabolism in the kidney | The natural turnover of MHC-I gives rise to the release of b2m into plasmatic fluids at ~0.1 um and to its catabolism in the | ||
In case of renal dysfunction, b2m concentration increases up to 60-fold, giving rise to pathogenic accumulation of filamentous structures, displaying the typical properties of amyloid fibrils, principally in the joints and connective tissue. | kidney. In case of renal dysfunction, b2m concentration increases up to 60-fold, giving rise to pathogenic accumulation of filamentous structures, displaying the typical properties of amyloid fibrils, principally in the joints and connective tissue. | ||
{{STRUCTURE_1duz | PDB=1duz | SCENE= }} | |||
==Monomeric human b2m== | |||
The first crystal structure of monomeric human b2m (Mhb2m) is solved in 2002. The protein is 99 residue in length and has a | |||
seven-stranded β sandwich fold typical of the Immunoglobulin superfamily. β strands A,B,D and E comprise one β sheet, and | |||
whereas β strands C,F,G form the second β sheet. The protein is stabilized by a single disulfide bond between Cys-25 and Cys-80, | |||
which links the two β sheets. | |||
