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Abstract
Rapidly progressive biopsy-proved renal amyloidosis developed in three brothers, aged
49, 52, and 55, of Irish-American origin. None had evidence of a plasma cell dyscrasia,
a monoclonal serum or urine protein, or any underlying chronic disease, immunoperoxidase
staining of one pulmonary and one renal biopsy specimen was negative for Amyloid A
(AA), Amyloid L (AL), and prealbumin. To investigate factors that might play a role
in the disease, the subjects and 21 relatives were typed for antigens of the A, B,
C, and DR loci and the linked marker genes for factor B and glyoxalase. The ability
of macrophages to degrade serum amyloid A (SAA) [1] was examined. One brother yielded
an intermediate AA-like product similar to what is seen in most patients with AA or
AL amytoldosis and 40 percent of normal subjects. The other two degraded SAA completely
to small peptldes. Analysis of the families revealed first that the disease was not
linked to the major histocompatibility complex. We were unable to demonstrate a genetic
relationship between processing of SAA by peripheral mononuclear cells and the human
leukocyte antigen locus. Finally, the pattern of SAA degradation was not associated
with the development of the disease.
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References
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Article info
Publication history
Accepted:
January 20,
1982
Footnotes
☆This work was supported by USPHS Research Grant AM 02594 and the Irvington House Institute.
Identification
Copyright
© 1982 Published by Elsevier Inc.
