Cellular Response of Cardiac Fibroblasts to Amyloidogenic Light Chains
نویسندگان
چکیده
منابع مشابه
Effect of lysine modification on the stability and cellular binding of human amyloidogenic light chains.
AL amyloidosis is characterized by the pathologic deposition as fibrils of monoclonal light chains (i.e., Bence Jones proteins [BJPs]) in particular organs and tissues. This phenomenon has been attributed to the presence in amyloidogenic proteins of particular amino acids that cause these molecules to become unstable, as well as post-translational modifications and, in regard to the latter, we ...
متن کاملRole of mutations in the cellular internalization of amyloidogenic light chains into cardiomyocytes
Light chain (AL) amyloidosis is characterized by the misfolding of immunoglobulin light chains, accumulating as amyloid fibrils in vital organs. Multiple reports have indicated that amyloidogenic light chains internalize into a variety of cell types, but these studies used urine-derived proteins without indicating any protein sequence information. As a result, the role of somatic mutations in a...
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Primary amyloidosis is a systemic disorder characterized by the clonal production and tissue deposition of immunoglobulin light chain (LC) proteins. Congestive heart failure remains the greatest cause of death in primary amyloidosis, due to the development of a rapidly progressive amyloid cardiomyopathy. Amyloid cardiomyopathy is largely unresponsive to current heart failure therapies, and is a...
متن کاملEvidence that amyloidogenic light chains undergo antigen-driven selection.
AL amyloidosis is characterized by fibrillar tissue deposits (amyloid) composed of monoclonal light chains secreted by small numbers of indolent bone marrow plasma cells whose ontogenesis is unknown. To address this issue and to provide insights into the processes that accompanied pathogenic light chain formation, we isolated the complete variable (V) regions of 14 light (VL) and 3 heavy (VH) c...
متن کاملDissociation of light chains from cardiac myosin.
The substrate, ATP, protects the active site of cardiac myosin during a 10-min treatment at 37 "C and neutral pH in the absence of divalent cations; under these conditions there is an approximate 20 % dissociation of light chain C1 and 60 % loss of light chain CZ with no corresponding decrease in myosin ATPase activity. Higher temperatures, the absence of divalent cations, increased treatment t...
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ژورنال
عنوان ژورنال: The American Journal of Pathology
سال: 2005
ISSN: 0002-9440
DOI: 10.1016/s0002-9440(10)62244-4