Chapter title |
β(2)-Microglobulin Amyloidosis.
|
---|---|
Chapter number | 19 |
Book title |
Protein Aggregation and Fibrillogenesis in Cerebral and Systemic Amyloid Disease
|
Published in |
Sub cellular biochemistry, December 2012
|
DOI | 10.1007/978-94-007-5416-4_19 |
Pubmed ID | |
Book ISBNs |
978-9-40-075415-7, 978-9-40-075416-4
|
Authors |
Corlin DB, Heegaard NH, Dorthe B Corlin, Niels H. H. Heegaard, Corlin, Dorthe B, Heegaard, Niels H. H. |
Abstract |
Dialysis-related amyloidosis (DRA) is a clinical syndrome of pain, loss of function and other symptoms due to the deposition of amyloid consisting of β(2)-microglobulin (β(2)m) in the musculoskeletal system. The condition is seen in patients who suffer from chronic kidney disease and are treated with hemodialysis for a long time. Even though β(2)m easily can be manipulated to form amyloid in laboratory experiments under non-physiological conditions the precise mechanisms involved in the formation of β(2)m-amyloid in patients with DRA have been difficult to unravel. The current knowledge which is reviewed here indicates that conformational fluctuations centered around the D-strand, the DE-loop, and around the cis-configured Pro32 peptide bond are involved in β(2)m amyloidosis. Also required are highly increased concentrations of circulating β(2)m and possibly various post-translational modifications mediated by the pro-inflammatory environment in uremic blood, together with the influence of divalent metal ions (specifically Cu(2 +)), uremic toxins, and dialysis-enhanced redox-processes. It seems plausible that domain-swapped β(2)m dimers act as building blocks of β-spine cross-β -sheet fibrils consisting of otherwise globular, roughly natively folded protein. An activated complement system and cellular activation perpetuate these reactions which due to the affinity of β(2)m-amyloid for the collagen of synovial surfaces result in the DRA syndrome. |
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