Search Results - "Connors, Lawreen H"
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Blood Proteomic Profiling in Inherited (ATTRm) and Acquired (ATTRwt) Forms of Transthyretin-Associated Cardiac Amyloidosis
Published in Journal of proteome research (07-04-2017)“…Transthyretin-associated forms of cardiac amyloidosis are fatal protein misfolding diseases that can be inherited (ATTRm) or acquired (ATTRwt). An accurate…”
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Monoclonal gammopathy of undetermined significance in systemic transthyretin amyloidosis (ATTR)
Published in Amyloid (02-01-2018)“…Objective: To identify the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients with transthyretin (ATTR) amyloidosis. Patients…”
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Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study
Published in Blood cancer journal (New York) (04-08-2021)“…The recent decades have ushered in considerable advancements in the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes…”
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Human amyloidogenic light chain proteins result in cardiac dysfunction, cell death, and early mortality in zebrafish
Published in American journal of physiology. Heart and circulatory physiology (01-07-2013)“…Systemic amyloid light-chain (AL) amyloidosis is associated with rapidly progressive and fatal cardiomyopathy resulting from the direct cardiotoxic effects of…”
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5
Features of atrial fibrillation in wild‐type transthyretin cardiac amyloidosis: a systematic review and clinical experience
Published in ESC Heart Failure (01-10-2018)“…Aims Wild‐type transthyretin (ATTRwt) cardiac amyloidosis has emerged as an important cause of heart failure in the elderly. Atrial fibrillation (AF) commonly…”
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6
Doxycycline reduces fibril formation in a transgenic mouse model of AL amyloidosis
Published in Blood (15-12-2011)“…Systemic AL amyloidosis results from the aggregation of an amyloidogenic immunoglobulin (Ig) light chain (LC) usually produced by a plasma cell clone in the…”
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Unusual duplication mutation in a surface loop of human transthyretin leads to an aggressive drug-resistant amyloid disease
Published in Proceedings of the National Academy of Sciences - PNAS (10-07-2018)“…Transthyretin (TTR) is a globular tetrameric transport protein in plasma. Nearly 140 single amino acid substitutions in TTR cause life-threatening amyloid…”
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Cardiac amyloidosis in African Americans: Comparison of clinical and laboratory features of transthyretin V122I amyloidosis and immunoglobulin light chain amyloidosis
Published in The American heart journal (01-10-2009)“…Background Transthyretin (TTR) mutations known to cause cardiac amyloidosis include V122I, found almost exclusively in African Americans at a prevalence of…”
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Lysosomal dysfunction and impaired autophagy underlie the pathogenesis of amyloidogenic light chain‐mediated cardiotoxicity
Published in EMBO molecular medicine (01-11-2014)“…AL amyloidosis is the consequence of clonal production of amyloidogenic immunoglobulin light chain (LC) proteins, often resulting in a rapidly progressive and…”
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Design and Rationale the SCAN-MP (Screening for Cardiac Amyloidosis With Nuclear Imaging in Minority Populations) Study
Published in Journal of the American Heart Association (18-04-2023)“…Background Transthyretin amyloid cardiomyopathy (ATTR-CM) is an important cause of heart failure in older individuals. Misfolding and deposition of…”
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Clinical Penetrance of the Transthyretin V122I Variant in Older Black Patients With Heart Failure: The SCAN-MP (Screening for Cardiac Amyloidosis With Nuclear Imaging in Minority Populations) Study
Published in Journal of the American Heart Association (01-08-2023)“…Background Transthyretin amyloid cardiomyopathy (ATTR-CM) is an underdiagnosed cause of heart failure (HF) among patients ≥60 years of age. Although the V122I…”
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12
Human Amyloidogenic Light Chains Directly Impair Cardiomyocyte Function Through an Increase in Cellular Oxidant Stress
Published in Circulation research (30-04-2004)“…Primary amyloidosis is a systemic disorder characterized by the clonal production and tissue deposition of immunoglobulin light chain (LC) proteins. Congestive…”
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The Critical Role of the Constant Region in Thermal Stability and Aggregation of Amyloidogenic Immunoglobulin Light Chain
Published in Biochemistry (Easton) (16-11-2010)“…Light chain (LC) amyloidosis (AL) is a fatal disease in which immunoglobulin LC deposit as fibrils. Although the LC amyloid-forming propensity is attributed…”
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14
Amyloidogenic and Associated Proteins in Systemic Amyloidosis Proteome of Adipose Tissue
Published in Molecular & cellular proteomics (01-08-2008)“…In systemic amyloidoses, widespread deposition of protein as amyloid causes severe organ dysfunction. It is necessary to discriminate among the different forms…”
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Cooperative Stabilization of Transthyretin by Clusterin and Diflunisal
Published in Biochemistry (Easton) (20-01-2015)“…The circulating protein transthyretin (TTR) can unfold, oligomerize, and form highly structured amyloid fibrils that are deposited in tissues, causing organ…”
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Evidence for a Functional Role of the Molecular Chaperone Clusterin in Amyloidotic Cardiomyopathy
Published in The American journal of pathology (2011)“…Molecular chaperones, including the extracellular protein clusterin (CLU), play a significant role in maintaining proteostasis; they have a unique capacity to…”
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Role of Glycosaminoglycan Sulfation in the Formation of Immunoglobulin Light Chain Amyloid Oligomers and Fibrils
Published in The Journal of biological chemistry (26-11-2010)“…Primary amyloidosis (AL) results from overproduction of unstable monoclonal immunoglobulin light chains (LCs) and the deposition of insoluble fibrils in…”
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Identification of Transthyretin Cardiac Amyloidosis Using Serum Retinol-Binding Protein 4 and a Clinical Prediction Model
Published in JAMA cardiology (01-03-2017)“…Transthyretin cardiac amyloidosis (ATTR) is an underrecognized cause of heart failure (HF) in older individuals, owing in part to difficulty in diagnosis. ATTR…”
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Lysosomal dysfunction and impaired autophagy underlie the pathogenesis of amyloidogenic light chain‐mediated cardiotoxicity
Published in EMBO molecular medicine (01-05-2015)Get full text
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20
AL-Base: a visual platform analysis tool for the study of amyloidogenic immunoglobulin light chain sequences
Published in Amyloid (01-03-2009)“…AL-Base, a curated database of human immunoglobulin (Ig) light chain (LC) sequences derived from patients with AL amyloidosis and controls, is described, along…”
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