Results 41 to 50 of about 14,209 (224)

Inhibition by small-molecule ligands of formation of amyloid fibrils of an immunoglobulin light chain variable domain. [PDF]

, 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
Brumshtein, Boris, Cascio, Duilio, Eisenberg, David S, Esswein, Shannon R, Ly, Alan T, Phillips, Martin L, Salwinski, Lukasz, Sawaya, Michael R   +7 more
core   +1 more source

Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience

Haematologica, 2013
Amyloidosis of the gastrointestinal tract, with biopsy-proven disease, is rare. We reviewed a series of patients who presented with biopsy-proven gastrointestinal amyloidosis and report their clinical characteristics, treatments, and survival.
Andrew J. Cowan, Martha Skinner, David C. Seldin, John L. Berk, David R. Lichtenstein, Carl J. O'Hara, Gheorghe Doros, Vaishali Sanchorawala   +7 more
doaj   +1 more source

Amyloid light-chain deposition in a schwannoma

Interdisciplinary Neurosurgery, 2021
Systemic amyloid light-chain amyloidosis is a protein misfolding disorder characterized by extracellular deposition of amyloid fibrils derived from abnormal clonal immunoglobulin light chains in various organ systems.
Megan M. Jack, Brandon W. Smith, Christopher J. Klein, Taxiarchis Kourelis, Andrew L. Folpe, Robert J. Spinner, Ellen D. McPhail   +6 more
doaj   +1 more source

Venetoclax in Immunoglobulin Light Chain Amyloidosis: Is This the Beginning or the End? [PDF]

Clin Lymphoma Myeloma Leuk, 2019
Abstract AL Amyloidosis (AL) is a plasma cell disease marked by production and deposition of toxic free light chains in organs including the heart, kidney and nervous system. While treatment is generally borrowed from multiple myeloma (MM), patients typically have difficulty tolerating many of these drugs, namely the immunomodulatory drugs (IMiDs ...
Premkumar V, Comenzo R, Lentzsch S.
europepmc   +4 more sources

Biomarkers in Immunoglobulin Light Chain Amyloidosis

Klinicka Onkologie, 2017
Immunoglobulin light chain amyloidosis (AL amyloidosis - ALA) is a monoclonal gammopathy characterized by presence of aberrant plasma cells producing amyloidogenic immunoglobulin light chains. This leads to formation of amyloid fibrils in various organs and tissues, mainly in heart and kidney, and causes their dysfunction.
Agnieszka Barchnicka, Jana Filipova, Tereza Sevcikova, Zuzana Kufova, Pavel Němec, Zdeněk Adam, Petr Vojta, Michal Simicek, Marian Hajduch, Kateřina Growková, Miroslav Penka, Vychytilova P, Lucie Brozova, Roman Hájek, Artur Jurczyszyn, Sebastian Grosicki, Luděk Pour, Romana Rysava   +17 more
openaire   +4 more sources

Light‐chain amyloid myopathy isolated to skeletal muscles: A case report

Clinical Case Reports, 2020
Isolated amyloidosis, especially of amyloid light‐chain type, is an infrequent disease. Systemic chemotherapy for light‐chain amyloidosis isolated to skeletal muscles plays a key role to reduce clonal plasma cells producing aberrant immunoglobulin.
Toshihiro Matsukawa, Katsuki Eguchi, Ichizo Nishino, Kohei Okada, Kazuo Oshimi, Takuto Miyagishima   +5 more
doaj   +1 more source

Chronic myopathy due to immunoglobulin light chain amyloidosis [PDF]

Molecular Genetics and Metabolism, 2013
Amyloid myopathy associated with a plasma cell dyscrasia is a rare cause of muscle hypertrophy. It can be a challenging diagnosis, since pathological findings are often elusive. In addition, the mechanism by which immunoglobulin light-chain deposition stimulates muscle overgrowth remains poorly understood. We present a 53-year old female with a 10-year
Manoli, Irini, Kwan, Justin Y, Wang, Qian, Rushing, Elisabeth J, Tsokos, Maria, Arai, Andrew E, Burch, Warner M, Dispenzieri, Angela, McPherron, Alexandra C, Gahl, William A   +9 more
openaire   +3 more sources

Three-dimensional structure of an immunoglobulin light-chain dimer with amyloidogenic properties [PDF]

, 2002
The X-ray structure of an immunoglobulin light-chain dimer isolated from the urine as a 'Bence-Jones protein' from a patient with multiple myeloma and amyloidosis (Sea) was determined at 1.94 Angstrom resolution and refined to R and R-free factors of 0 ...
Bourne, P. C., DeWitt, C. R., Edmundson, A. B., Fan, Z. C., Guddat, L. W., Moomaw, C. R., Ramsland, P. A., Shan, L., Shultz, B. B., Slaughter, C. A., Terzyan, S. S.   +10 more
core   +2 more sources

Monoclonal gammopathy of renal significance: Diagnostic workup [PDF]

, 2017
The clinical spectrum of diseases associated with monoclonal gammopathies is wide and they are most commonly the consequence of renal deposition of monoclonal immunoglobulin or its components.
Cabrita, A., Correia, S., Malheiro, J., Martins, L., Santos, J., Santos, S.   +5 more
core   +1 more source

Cardiac Amyloidosis – An Underdiagnosed Cause of Heart Failure with Preserved Ejection Fraction – Updated Diagnosis and Treatment Options [PDF]

Romanian Journal of Cardiology, 2021
Cardiac amyloidosis (CA) still represents a frequently missed cause of heart failure with preserved ejection fraction (HFpEF). In the light of many new and effective therapies for immunoglobulin light chain amyloidosis (AL) and for transthyretin ...
Roxana Cristina RIMBAS, Anca BALINISTEANU, Alexandra Maria CHITROCEANU, Dragos VINEREANU   +3 more
doaj   +1 more source