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 …

Misfolded protein oligomers: mechanisms of formation, cytotoxic effects, and pharmacological approaches against protein misfolding diseases

DJ Rinauro, F Chiti, M Vendruscolo… - Molecular …, 2024 - Springer
The conversion of native peptides and proteins into amyloid aggregates is a hallmark of over
50 human disorders, including Alzheimer's and Parkinson's diseases. Increasing evidence …

Cryo-EM structure of cardiac amyloid fibrils from an immunoglobulin light chain AL amyloidosis patient

P Swuec, F Lavatelli, M Tasaki, C Paissoni… - Nature …, 2019 - nature.com
Systemic light chain amyloidosis (AL) is a life-threatening disease caused by aggregation
and deposition of monoclonal immunoglobulin light chains (LC) in target organs. Severity of …

How I treat AL amyloidosis

G Palladini, G Merlini - Blood, The Journal of the American …, 2022 - ashpublications.org
The treatment of patients with systemic light chain (AL) amyloidosis is a challenge to
hematologists. Despite its generally small size, the underlying clone causes a rapidly …

AL amyloidosis: from molecular mechanisms to targeted therapies

G Merlini - Hematology 2014, the American Society of …, 2017 - ashpublications.org
Systemic amyloidosis is caused by misfolding and extracellular deposition of circulating
proteins as amyloid fibrils, resulting in the dysfunction of vital organs. The most common …

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 …

Amyloid seeding of transthyretin by ex vivo cardiac fibrils and its inhibition

L Saelices, K Chung, JH Lee, W Cohn… - Proceedings of the …, 2018 - National Acad Sciences
Each of the 30 human amyloid diseases is associated with the aggregation of a particular
precursor protein into amyloid fibrils. In transthyretin amyloidosis (ATTR), mutant or wild-type …

Proteotoxicity in cardiac amyloidosis: amyloidogenic light chains affect the levels of intracellular proteins in human heart cells

E Imperlini, M Gnecchi, P Rognoni, E Sabidò… - Scientific reports, 2017 - nature.com
AL amyloidosis is characterized by widespread deposition of immunoglobulin light chains
(LCs) as amyloid fibrils. Cardiac involvement is frequent and leads to life-threatening …

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 …