Widespread amyloidogenicity potential of multiple myeloma patient-derived immunoglobulin light chains [PDF]
BMC Biology, 2023 Background In a range of human disorders such as multiple myeloma (MM), immunoglobulin light chains (IgLCs) can be produced at very high concentrations. This can lead to pathological aggregation and deposition of IgLCs in different tissues, which in turn
Rebecca Sternke-Hoffmann +19 more
doaj +2 more sources
Mechanistic Insights into the Early Events in the Aggregation of Immunoglobulin Light Chains. [PDF]
Biochemistry, 2019 Little is known about the mechanism of amyloid assembly in immunoglobulin light chain (AL) amyloidosis, in contrast to other amyloid diseases. Early events in the aggregation pathway are especially important, as these soluble species could be cytotoxic ...
Misra P +2 more
europepmc +2 more sources
Immunoglobulin light chains generate proinflammatory and profibrotic kidney injury. [PDF]
J Clin Invest, 2019 Because of the less-than-robust response to therapy and impact on choice of optimal chemotherapy and prognosis, chronic kidney disease has drawn attention in the treatment of multiple myeloma, a malignant hematologic disorder that can produce significant
Ying WZ +5 more
europepmc +2 more sources
Inhibition by small-molecule ligands of formation of amyloid fibrils of an immunoglobulin light chain variable domain [PDF]
eLife, 2015 Overproduction of immunoglobulin light chains leads to systemic amyloidosis, a lethal disease characterized by the formation of amyloid fibrils in patients' tissues. Excess light chains are in equilibrium between dimers and less stable monomers which can
Boris Brumshtein +7 more
doaj +3 more sources
Stabilization of amyloidogenic immunoglobulin light chains by small molecules. [PDF]
Proc Natl Acad Sci U S A, 2019 Significance Light-chain amyloidosis (AL) is a progressive and often fatal degenerative disease caused by conformational changes within Ig light chains that generally lead to aggregation. Current chemotherapy treatments aim to eliminate the clonal plasma
Morgan GJ +15 more
europepmc +2 more sources
The CDR1 and Other Regions of Immunoglobulin Light Chains are Hot Spots for Amyloid Aggregation. [PDF]
Sci Rep, 2019 Immunoglobulin light chain-derived (AL) amyloidosis is a debilitating disease without known cure. Almost nothing is known about the structural factors driving the amyloidogenesis of the light chains.
Ruiz-Zamora RA +13 more
europepmc +2 more sources
Systemic immunoglobulin light chain amyloidosis [PDF]
Nature Reviews Disease Primers, 2018 Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction.
G. Merlini +6 more
semanticscholar +7 more sources
Role of domain interactions in the aggregation of full-length immunoglobulin light chains. [PDF]
Proc Natl Acad Sci U S A, 2019 Significance The misfolding and/or aggregation of proteins can give rise to amyloid diseases, including immunoglobulin light-chain (LC) amyloidosis, leading to large proteinaceous deposits in organs throughout the body.
Rennella E, Morgan GJ, Kelly JW, Kay LE.
europepmc +2 more sources
Concurrent structural and biophysical traits link with immunoglobulin light chains amyloid propensity. [PDF]
Sci Rep, 2017 Light chain amyloidosis (AL), the most common systemic amyloidosis, is caused by the overproduction and the aggregation of monoclonal immunoglobulin light chains (LC) in target organs.
Oberti L +9 more
europepmc +2 more sources
Daratumumab-Based Treatment for Immunoglobulin Light-Chain Amyloidosis. [PDF]
New England Journal of Medicine, 2021 BACKGROUND Systemic immunoglobulin light-chain (AL) amyloidosis is characterized by deposition of amyloid fibrils of light chains produced by clonal CD38+ plasma cells. Daratumumab, a human CD38-targeting antibody, may improve outcomes for this disease.
E. Kastritis +49 more
semanticscholar +12 more sources