systemic amyloidoses

systemic amyloidoses

A group of diseases that have in common the deposition, outside the cells, of an abnormal, insoluble fibrillary protein called amyloid. In primary AMYLOIDOSIS the amyloid fibrils are monoclonal fragments of antibody (immunoglobulin) light chains. In secondary and familial amyloidoses other proteins, including apolipoprotein, fibrinogen, lysozyme and transthyretin, are involved.
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The broad applicability of the GAIM technology enables the company to target multiple protein misfolding diseases, including neurodegenerative diseases and orphan systemic amyloidoses.
4,55,56,59-80) Typing the most common systemic amyloidoses (ie, AA, AL, AApol, Afib, ALys, and ATTR amyloid) could be done in the kidney biopsies.
In Europe, approximately 45% of systemic amyloidoses are AA amyloidosis.
Although most of the systemic amyloidoses affect the kidney, there is significant variability in the degree of renal involvement between patients.
Hereditary systemic amyloidoses with renal involvement.
The broad applicability of the Proclara technology enables the company to target multiple protein misfolding diseases, including neurodegenerative diseases and several rare systemic amyloidoses.
However, recent advances in the treatment of systemic amyloidoses have changed this outlook and, hence, the importance of an early diagnosis of amyloid, and a correct diagnosis of its type, has been realized.
Although, at present, management of localized amyloid deposits is mainly conservative, treatment of systemic amyloidoses involves radical approaches ranging from aggressive chemotherapy to liver transplantation; new pharmacologic therapies targeting systemic fibrillogenesis are also in trials.
The role of genetics in systemic amyloidoses has recently been reevaluated.
Among systemic amyloidoses there are 3 sporadic forms and several familial forms.
Other hereditary systemic amyloidoses, including AApoAI, AGel, ALys, and AFib, are relatively rare.
These compounds include amyloid P component (AP; glycoprotein related to C-reactive protein), apolipoprotein E (Apo-E; regulator of lipoprotein metabolism, Figure 5), and sulfated glycosaminoglycans (GAGs; constituents of matrix proteins)[41,42] In contrast, [Alpha]1-antichymotrypsin (a serine protease inhibitor, Figure 6) has been detected only in A[Beta], whereas the deposits present in systemic amyloidoses have been shown to be negative.

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