Systemic immunoglobulin light chain amyloidosis

G Merlini, A Dispenzieri, V Sanchorawala… - Nature reviews Disease …, 2018 - nature.com
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 …

Protein aggregation–mechanisms, detection, and control

W Wang, CJ Roberts - International journal of pharmaceutics, 2018 - Elsevier
Protein aggregation has been recognized as one of the major challenges in the
development and commercialization of successful protein-based drug products because of …

Dissection of the amyloid formation pathway in AL amyloidosis

P Kazman, RM Absmeier, H Engelhardt… - Nature …, 2021 - nature.com
In antibody light chain (AL) amyloidosis, overproduced light chain (LC) fragments
accumulate as fibrils in organs and tissues of patients. In vitro, AL fibril formation is a slow …

Science and art of protein formulation development

W Wang, S Ohtake - International journal of pharmaceutics, 2019 - Elsevier
Protein pharmaceuticals have become a significant class of marketed drug products and are
expected to grow steadily over the next decade. Development of a commercial protein …

Immunoglobulin light chain amyloid aggregation

LM Blancas-Mejia, P Misra, CJ Dick… - Chemical …, 2018 - pubs.rsc.org
Light chain (AL) amyloidosis is a devastating, complex, and incurable protein misfolding
disease. It is characterized by an abnormal proliferation of plasma cells (fully differentiated B …

An N-glycosylation hotspot in immunoglobulin κ light chains is associated with AL amyloidosis

A Nevone, M Girelli, S Mangiacavalli, B Paiva, P Milani… - Leukemia, 2022 - nature.com
Immunoglobulin light chain (AL) amyloidosis is caused by a small, minimally proliferating B-
cell/plasma-cell clone secreting a patient-unique, aggregation-prone, toxic light chain (LC) …

The kinetic stability of a full-length antibody light chain dimer determines whether endoproteolysis can release amyloidogenic variable domains

GJ Morgan, JW Kelly - Journal of molecular biology, 2016 - Elsevier
Light chain (LC) amyloidosis (AL amyloidosis) appears to be caused by the misfolding, or
misfolding and aggregation of an antibody LC or fragment thereof and is fatal if untreated …

Stabilization of amyloidogenic immunoglobulin light chains by small molecules

GJ Morgan, NL Yan, DE Mortenson… - Proceedings of the …, 2019 - National Acad Sciences
In Ig light-chain (LC) amyloidosis (AL), the unique antibody LC protein that is secreted by
monoclonal plasma cells in each patient misfolds and/or aggregates, a process leading to …

Fatal amyloid formation in a patient's antibody light chain is caused by a single point mutation

P Kazman, MT Vielberg, MD Pulido Cendales… - Elife, 2020 - elifesciences.org
In systemic light chain amyloidosis, an overexpressed antibody light chain (LC) forms fibrils
which deposit in organs and cause their failure. While it is well-established that mutations in …

Concurrent structural and biophysical traits link with immunoglobulin light chains amyloid propensity

L Oberti, P Rognoni, A Barbiroli, F Lavatelli, R Russo… - Scientific reports, 2017 - nature.com
Light chain amyloidosis (AL), the most common systemic amyloidosis, is caused by the
overproduction and the aggregation of monoclonal immunoglobulin light chains (LC) in …