scholarly journals The effects of sodium sulfate, glycosaminoglycans, and Congo red on the structure, stability, and amyloid formation of an immunoglobulin light-chain protein

2006 ◽  
Vol 15 (7) ◽  
pp. 1710-1722 ◽  
Author(s):  
Richard W. Mclaughlin ◽  
Janelle K. De Stigter ◽  
Laura A. Sikkink ◽  
Elizabeth M. Baden ◽  
Marina Ramirez-Alvarado
Keyword(s):  
Light Chain ◽  
Congo Red ◽  
Sodium Sulfate ◽  
Structure Stability ◽  
Amyloid Formation ◽  
Immunoglobulin Light Chain

scholarly journals Heat-induced native dimerization prevents amyloid formation by variable domain from immunoglobulin light-chain REI

FEBS Journal ◽  
2017 ◽  
Vol 284 (18) ◽  
pp. 3114-3127 ◽  
Author(s):  
Marina Nawata ◽  
Hirotaka Tsutsumi ◽  
Yuta Kobayashi ◽  
Satoru Unzai ◽  
Shouhei Mine ◽  
...  
Keyword(s):  
Light Chain ◽  
Variable Domain ◽  
Amyloid Formation ◽  
Immunoglobulin Light Chain

Frequency of Amyloid Subtyping Among Patients with Immunoglobulin Light-Chain Amyloidosis Referred for High-Dose Chemotherapy and Autologous Stem Cell Transplant

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5601-5601
Author(s):  
Andrew J. Cowan ◽  
David G. Coffey ◽  
Teresa S. Hyun ◽  
Pamela S. Becker ◽  
Damian J. Green ◽  
...  
Keyword(s):  
Mass Spectrometry ◽  
Plasma Cell ◽  
Light Chain ◽  
Congo Red ◽  
Plasma Cell Dyscrasia ◽  
High Dose ◽  
Al Amyloidosis ◽  
Cell Transplant ◽  
Immunoglobulin Light Chain ◽  
Organ Systems

Abstract Background: The amyloidoses comprise a heterogeneous group of diseases characterized by misfolding of amyloidogenic proteins and subsequent deposition as amyloid fibrils. To date, over 30 proteins are known to be amyloidogenic (Sipe Amyloid 2014). Immunoglobulin light chain (AL) amyloidosis, a plasma cell dyscrasia, is the most common subtype. The standard diagnostic algorithm in AL amyloidosis is to obtain a biopsy of a clinically involve organ, and once Congo red positivity is confirmed, perform subtyping analyses with immunohistochemistry or mass spectrometry. Accurate subtyping of amyloidosis is essential to appropriate treatment, as misdiagnosis occurs in up to 10% of patients and may lead to inappropriate administration of chemotherapy (Comenzo Blood 2006; Lachmann NEJM 2002). We sought to determine the patterns of amyloid subtyping among patients with a diagnosis of AL amyloidosis referred to a tertiary referral center for HDM/SCT. Methods: Sequential patients with confirmed amyloidosis, age ≥ 18 years who underwent HDM/SCT between 2001 and 2014 at the Fred Hutchinson Cancer Research Center and University of Washington Medical Center were eligible. Presence of a Congo red-positive biopsy for each patient referred for transplant was confirmed and the pathology reports and medical records were reviewed to determine if subtyping was performed, and which modality was used. Results: Fifty-one patients with AL amyloidosis were referred for transplant; of these, 45 proceeded with HDM/SCT. The organ systems most commonly involved were renal in 34/51, and gastrointestinal in 5/51. Of the biopsies, subtyping was performed in 35 (68.6%), and no subtyping was performed in 16 patients (31.3%). Immunofluorescence was the most common modality used for subtyping in 33 biopsies (94.2%) and laser capture/mass spectrometry (LC/MS) was used in 2 patients (5.7%). All patients had evidence of a clonal plasma cell dyscrasia by bone marrow biopsy and peripheral blood testing. Of the patients without subtyping, 8 (50%) were diagnosed before 2008. Discussion: Misdiagnosis of amyloidosis due to a lack of appropriate subtyping is a well-described and ongoing problem for patients with amyloidosis. These data suggest that definitive subtyping is still not routinely performed in the evaluation of amyloidosis. At our center, efforts to standardize the evaluation of Congo-red positive biopsies using definitive typing are underway. Disclosures Gopal: Seattle Genetics: Research Funding.

Probing the role of λ6 immunoglobulin light chain dimerization in amyloid formation

2016 ◽  
Vol 1864 (4) ◽  
pp. 409-418 ◽  
Author(s):  
Mathieu Laporte Wolwertz ◽  
Phuong Trang Nguyen ◽  
Noé Quittot ◽  
Steve Bourgault
Keyword(s):  
Light Chain ◽  
Amyloid Formation ◽  
Immunoglobulin Light Chain

scholarly journals Systemic Light Chain Amyloidosis (Congo Red Inconclusive) with Underlyng Clonal Expansion Not Meeting the Criteria of Light Chain Monoclonal Gammopathy of Renal Significance or Light Chain Myeloma:A Case Report

2020 ◽  
Vol 07 (04) ◽  
pp. 15-19
Author(s):  
Sanjay Kumar ◽  
Keyword(s):  
Light Chain ◽  
Congo Red ◽  
Monoclonal Gammopathy ◽  
Plasma Cells ◽  
Clonal Expansion ◽  
Chronic Diarrhoea ◽  
Al Amyloidosis ◽  
Immunoglobulin Light Chain ◽  
Light Chain Amyloidosis ◽  
Immunoglobulin Light Chain Amyloidosis

Fifty-eight-year-old male admitted for evaluation of nephrotic syndrome and chronic diarrhoea was detected to have Immunoglobulin light chain amyloidosis (AL Amyloidosis) which was congo red inconclusive from renal biopsy. Bone marrow biopsy showed monoclonal plasma cells of 40% and light chain assay showed predominance of immunoglobulin lambda light chain. The diagnosis was neither fitting into the current diagnostic criteria for light chain Monoclonal Gammopathy of Renal Significance (MGRS) nor light chain myeloma. Literature is scarce regarding patients with AL amyloidosis having underlying clonal expansion not meeting the criteria of light chain myeloma or light chain MGRS.

scholarly journals Inhibition by small-molecule ligands of formation of amyloid fibrils of an immunoglobulin light chain variable domain

eLife ◽  
2015 ◽  
Vol 4 ◽  
Author(s):  
Boris Brumshtein ◽  
Shannon R Esswein ◽  
Lukasz Salwinski ◽  
Martin L Phillips ◽  
Alan T Ly ◽  
...  
Keyword(s):  
Small Molecule ◽  
Light Chain ◽  
Amyloid Fibrils ◽  
Variable Domain ◽  
Light Chains ◽  
Amyloid Formation ◽  
Immunoglobulin Light Chains ◽  
Immunoglobulin Light Chain ◽  
Small Molecule Ligands ◽  
Irreversible Aggregation

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 undergo irreversible aggregation to the amyloid state. The dimers therefore must disassociate into monomers prior to forming amyloid fibrils. Here we identify ligands that inhibit amyloid formation by stabilizing the Mcg light chain variable domain dimer and shifting the equilibrium away from the amyloid-prone monomer.

scholarly journals A Unique Case of Combined Nodular and Tracheobronchial Amyloidosis

2021 ◽  
Vol 2021 (2) ◽  
Author(s):  
Feihong Ding ◽  
Yun Li ◽  
Shailesh Balasubramanian ◽  
Subha Ghosh ◽  
Jason N Valent ◽  
...  
Keyword(s):  
Light Chain ◽  
Systemic Disease ◽  
Amyloid Deposition ◽  
Misfolded Proteins ◽  
Immunoglobulin Light Chain ◽  
Anatomic Site ◽  
Unique Case ◽  
Pulmonary Amyloidosis ◽  
Immunoglobulin Light Chain Amyloidosis ◽  
Tracheobronchial Amyloidosis

ABSTRACT Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of misfolded proteins that can affect either systemically or locally confined to one system. Pulmonary amyloidosis is rare and can be classified into three forms according to the anatomic site of involvement: nodular pulmonary amyloidosis, tracheobronchial amyloidosis and diffuse alveolar-septal amyloidosis. The former two usually represent localized amyloid disease and the latter represents systemic disease. Typically lung parenchymal and tracheobronchial amyloidosis do not present together in localized forms of pulmonary amyloidosis. Here we report a unique case of localized pulmonary immunoglobulin light-chain amyloidosis, manifested as both parenchymal nodules and tracheobronchial amyloid deposition.

scholarly journals Structure and Location of Protein Sites Binding Self-Associated Congo Red Molecules with Intercalated Drugs as Compact Ligands—Theoretical Studies

Biomolecules ◽  
2021 ◽  
Vol 11 (4) ◽  
pp. 501
Author(s):  
Ptak-Kaczor Magdalena ◽  
Kwiecińska Klaudia ◽  
Korchowiec Jacek ◽  
Chłopaś Katarzyna ◽  
Banach Mateusz ◽  
...  
Keyword(s):  
Organic Compounds ◽  
Light Chain ◽  
Congo Red ◽  
Theoretical Studies ◽  
Mathematical Tool ◽  
Supramolecular Systems ◽  
Micellar Structure ◽  
Protein Molecules ◽  
Toxic Drugs ◽  
Independent Property

In the search for new carriers capable of transporting toxic drugs to a target, particular attention has been devoted to supramolecular systems with a ribbon-like micellar structure of which Congo red is an example. A special promise of the possible use of such systems for directing drugs to a target emerges from their particular affinity to immune complexes and as an independent property, binding many organic compounds including drugs by intercalation. Serum albumin also appeared able to bind micellar particles of such systems. It may protect them against dilution in transport. The mathematical tool, which relies on analysis of the distribution of polarity and hydrophobicity in protein molecules (fuzzy oil drop model), has been used to find the location of binding area in albumin as well as anchorage site for Congo red in heated IgG light chain used as a model presenting immunoglobulin-like structures. Results confirm the suggested formerly binding site of Congo red in V domain of IgG light chain and indicated the cleft between pseudo-symmetric domains of albumin as the area of attachment for the dye.

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