Amyloid-related

A19. Anders, R. F., Natvig, J. B., Michaelsen, T. E., and Husby, G., Isolation and characterization of amyloid-related serum protein SAA as a low molecular weight protein. Scand. J. Immunol. 4, 397-401 (1975). 

E8. Eriksen, N., and Benditt, E. P., Isolation and characterization of the amyloid-related apoprotein (SAA) from human high density lipoprotein. Proc. Natl. Acad. Sci. U.S.A. 77, 6860-6864 (1980). 

M30. Marhaug, G., Sletten, K., and Husby, G., Characterization of amyloid-related protein SAA complexed with serum lipoproteins (apo SAA). Clin. Exp. Immunol. 50, 382-389... 

Schostarez (3) prepared aza-hydroxylethyl derivatives, (II), which were effective as (3-secretase enzyme inhibitors in the treatment of amyloid-related diseases. 

This intrinsic stickiness of unfolded polypeptides appears to be one of the causes of prion diseases and other amyloid-related conditions such as mad cow disease, CJD, Alzheimer s, and so forth. In such conditions, unfolded or misfolded proteins aggregate into lumps or plaques that interfere with normal cell function. 

Levin et al. were unable to find any amyloid of nonimmunoglobulin nature in the deposits of three patients with multiple myeloma or mac-roglobulinemia. However, the converse was not true, since some material related to L chain was found in patients with secondary amyloidosis, whose deposits were mainly nonimmunoglobulin in nature. This immunoglobulin-like material appeared heterogeneous, in contrast to that seen in myeloma patients. Relatively few individuals with secondary amyloidosis have been examined at this writing for the presence of amyloid related to immunoglobulin. 

Four cases of pleural effusion and biopsy-proven pleural amyloid infiltration in patients with AA amyloidosis have been reported in the past 30 years (17-19). Three cases occurred in patients with long-standing rheumatoid arthritis and the remaining patient had cystic fibrosis. No amyloid-related pleural effusions have been reported in FMF. The rarity of pleural effusions in AA amyloidosis is further evidenced by the absence of pleural disease at autopsy in 113 A A patients (14), a registry of 287 Turkish AA patients (36), or autopsies of 7 patients— despite bronchial waU or pulmonary vessel amyloid deposition in over 70% (13). 

Amyloid cardiomyopathy is extremely unusual in AA amyloidosis, emphasizing the importance of pleural disruption by amyloid deposition in the formation of pleural effusions. Given the paucity of reports documenting amyloid-related pleural disease in this population, pleural effusions in AA patients should generally be ascribed to other causes. 

MHC to exit the endoplasmic reticulum (ER). As a result, there is an endoplasmic reticulum stress response, which leads to activation of the transcription factor nuclear factor kB (NFkB) and further cytokine release with subsequent accumulation of misfoided MHC glycoproteins, including phosphorylated tau and amyloid-related proteins. (Reproduced fromDalakas [72].)... 

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