Amyloidosisisadisordercharacterizedbytheextracellulardepositsofproteinsthatarepronetoaggregateandforminsolublefibrils.
Thedepositionoftheseproteinsmayresultfrom:excessiveproductionofproteinsthatarepronetoaggregation;mutationsthatproduceproteinsthatcannotfoldproperlyandtendtoaggregate;defectiveorincompleteproteolyticdegradationofextracellularproteins.
Amyloidosismaybelocalizedorsystemic.Itisseeninassociationwithavarietyofprimarydisorders,includingmonoclonalB-cellproliferations(inwhichtheamyloiddepositsconsistofimmunoglobulinlightchains);chronicinflammatorydiseasessuchasrheumatoidarthritis(depositsofamyloidAprotein,derivedfromanacute-phaseproteinproducedininflammation);Alzheimerdisease(amyloidβ protein);familialconditionsinwhichtheamyloiddepositsconsistofmutatedproteins(e.g.,transthyretininfamilialamyloidpolyneuropathies);andhemodialysis(depositsofβ2-microglobulin,whoseclearanceisdefective).
Amyloiddepositscausetissueinjuryandimpairnormalfunctionbycausingpressureoncellsandtissues.Theydonotevokeaninflammatoryresponse.
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