Search Results to Lawreen Heller Connors, PhD

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Research Expertise & Professional Interests My research focuses on understanding the amyloidogenic nature of transthyretin (TTR), normally a soluble protein present in plasma and cerebral spinal fluid. Both wild-type and specific variant forms of TTR can be amyloidogenic, but disease onset is delayed in what appears to be an age-dependent mechanism. Our investigations are aimed at identifying specific age-related factors required to initiate the disease process; these factors likely include structural features that are both intrinsic and extrinsic to TTR. My research attempts to link protein biochemistry to disease pathology by studying the role of amino acid alterations, post-translational modifications (glycosylation, sulfonation, cysteinylation and phosphorylation) and metabolic processing in TTR amyloid fibril formation. We have precisely defined the molecular composition of numerous amyloidogenic TTR proteins derived from patient serum and tissue samples using mass spectrometry, analytical ultracentrifugation, and electron microscopy. In addition, using a proteomic approach, we have identified several tissue components present in patient specimens which may be effectors of amyloidogenesis. With recombinantly-generated proteins, we are studying the molecular stability of various forms of TTR, as well as the heteroassociations of TTR with these deposited molecular constituents using electrophoretic, chromatographic, and histological techniques. A translational component of our studies involves the study of a variety of compounds, including the small molecules diflunisal and a-tocopherol, as potential inhibitors of TTR aggregation and fibril formation. Furthermore, since TTR-associated amyloid diseases often feature cardiomyopathies, we are also studying the direct and indirect effects of amyloidogenic forms of TTR on cultured primary cardiac cells.
Self-Described Keywords cardiomyopathy

One or more keywords matched the following items that are connected to Connors, Lawreen

Item TypeName
Concept Amyloid
Concept Serum Amyloid A Protein
Concept Cardiomyopathies
Concept Amyloid beta-Protein Precursor
Concept Amyloid Neuropathies, Familial
Concept Plaque, Amyloid
Academic Article A new transthyretin variant (Ser23Asn) associated with familial amyloidosis in a Portuguese patient.
Academic Article A new amyloidogenic transthyretin variant (Val122Ala) found in a compound heterozygous patient.
Academic Article Tabulation of transthyretin (TTR) variants as of 1/1/2000.
Academic Article Betabellins 15D and 16D, de Novo designed beta-sandwich proteins that have amyloidogenic properties.
Academic Article Biophysical analysis of normal transthyretin: implications for fibril formation in senile systemic amyloidosis.
Academic Article Identification of a novel transthyretin Thr59Lys/Arg104His. A case of compound heterozygosity in a Chinese patient diagnosed with familial transthyretin amyloidosis.
Academic Article Transthyretin amyloidosis associated with a novel variant (Trp41Leu) presenting with vitreous opacities.
Academic Article Tabulation of human transthyretin (TTR) variants, 2003.
Academic Article Human amyloidogenic light chains directly impair cardiomyocyte function through an increase in cellular oxidant stress.
Academic Article A rare transthyretin mutation (Asp18Glu) associated with cardiomyopathy.
Academic Article Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis.
Academic Article Quantitative serum free light chain assay in the diagnostic evaluation of AL amyloidosis.
Academic Article Role of endocytic inhibitory drugs on internalization of amyloidogenic light chains by cardiac fibroblasts.
Academic Article Detailed structural analysis of amyloidogenic wild-type transthyretin using a novel purification strategy and mass spectrometry.
Academic Article A new transthyretin variant (Glu61Gly) associated with cardiomyopathy.
Academic Article Serum transthyretin levels in senile systemic amyloidosis: effects of age, gender and ethnicity.
Academic Article Amyloidogenic and associated proteins in systemic amyloidosis proteome of adipose tissue.
Academic Article AL-Base: a visual platform analysis tool for the study of amyloidogenic immunoglobulin light chain sequences.
Academic Article Cardiac amyloidosis in African Americans: comparison of clinical and laboratory features of transthyretin V122I amyloidosis and immunoglobulin light chain amyloidosis.
Academic Article Matrix metalloproteinases and their tissue inhibitors in cardiac amyloidosis: relationship to structural, functional myocardial changes and to light chain amyloid deposition.
Academic Article Amyloidogenic light chains induce cardiomyocyte contractile dysfunction and apoptosis via a non-canonical p38alpha MAPK pathway.
Academic Article Role of glycosaminoglycan sulfation in the formation of immunoglobulin light chain amyloid oligomers and fibrils.
Academic Article Spontaneous rupture of the liver in a patient with systemic AL amyloidosis undergoing treatment with high-dose melphalan and autologous stem cell transplantation: a case report with literature review.
Academic Article Isolation and characterization of a kappa amyloid fibril protein.
Academic Article Evidence for a functional role of the molecular chaperone clusterin in amyloidotic cardiomyopathy.
Academic Article Preclinical development of siRNA therapeutics for AL amyloidosis.
Academic Article Doxycycline reduces fibril formation in a transgenic mouse model of AL amyloidosis.
Academic Article Dysregulation of miRNAs in AL amyloidosis.
Academic Article Metabolic phenotype in an AL amyloidosis transgenic mouse model.
Academic Article High-dose melphalan and autologous stem cell transplantation for AL amyloidosis: recent trends in treatment-related mortality and 1-year survival at a single institution.
Academic Article Clinical features and survival in senile systemic amyloidosis: comparison to familial transthyretin cardiomyopathy.
Academic Article Detection of high-molecular-weight amyloid serum protein complexes using biological on-line tracer sedimentation.
Academic Article A new lysozyme tyr54asn mutation causing amyloidosis in a family of Swedish ancestry with gastrointestinal symptoms.
Academic Article Circulating matrix metalloproteinases and tissue inhibitors of metalloproteinases in cardiac amyloidosis.
Academic Article Human amyloidogenic light chain proteins result in cardiac dysfunction, cell death, and early mortality in zebrafish.
Academic Article Stanniocalcin1 is a key mediator of amyloidogenic light chain induced cardiotoxicity.
Academic Article In vitro formation of amyloid fibrils from intact beta 2-microglobulin.
Academic Article The pathogenesis and biochemistry of amyloidosis.
Academic Article The association of an elastase with amyloid fibrils.
Academic Article Nephelometric measurement of human serum prealbumin and correlation with acute-phase proteins CRP and SAA: results in familial amyloid polyneuropathy.
Academic Article Selective binding of nifedipine to amyloid fibrils.
Academic Article Lowered prealbumin levels in patients with familial amyloid polyneuropathy (FAP) and their non-affected but at risk relatives.
Academic Article Observations on the amyloid-degrading activity of serum and its relationship to human neutrophil elastase.
Academic Article Homozygosity for the V122I mutation in transthyretin is associated with earlier onset of cardiac amyloidosis in the African American population in the seventh decade of life.
Academic Article Transthyretin aggregate-specific antibodies recognize cryptic epitopes on patient-derived amyloid fibrils.
Academic Article Cooperative stabilization of transthyretin by clusterin and diflunisal.
Academic Article Vertebral compression fractures as the initial presentation of AL amyloidosis: case series and review of literature.
Academic Article Genetic variation of the transthyretin gene in wild-type transthyretin amyloidosis (ATTRwt).
Academic Article Heart Failure Resulting From Age-Related Cardiac Amyloid Disease Associated With Wild-Type Transthyretin: A Prospective, Observational Cohort Study.
Academic Article Immunoglobulin heavy light chain test quantifies clonal disease in patients with AL amyloidosis and normal serum free light chain ratio.
Academic Article Retinol binding protein 4 (RBP4) concentration identifies V122I transthyretin cardiac amyloidosis.
Academic Article Identification of Transthyretin Cardiac Amyloidosis Using Serum Retinol-Binding Protein 4 and a Clinical Prediction Model.
Academic Article Blood Proteomic Profiling in Inherited (ATTRm) and Acquired (ATTRwt) Forms of Transthyretin-Associated Cardiac Amyloidosis.
Academic Article Familial wild-type transthyretin cardiomyopathy.
Academic Article In vitro co-expression of human amyloidogenic immunoglobulin light and heavy chain proteins: a relevant cell-based model of AL amyloidosis.
Academic Article Serum Proteomic Variability Associated with Clinical Phenotype in Familial Transthyretin Amyloidosis (ATTRm).
Academic Article Prevalence of mutant ATTR cardiac amyloidosis in elderly African Americans with heart failure.
Academic Article Monoclonal gammopathy of undetermined significance in systemic transthyretin amyloidosis (ATTR).
Academic Article Use of Serum Transthyretin as a Prognostic Indicator and Predictor of Outcome in Cardiac Amyloid Disease Associated With Wild-Type Transthyretin.
Academic Article Features of atrial fibrillation in wild-type transthyretin cardiac amyloidosis: a systematic review and clinical experience.
Academic Article Unusual duplication mutation in a surface loop of human transthyretin leads to an aggressive drug-resistant amyloid disease.
Academic Article A library of ATTR amyloidosis patient-specific induced pluripotent stem cells for disease modelling and in vitro testing of novel therapeutics.
Academic Article Alterations in the secondary structure of mutant transthyretins associated with familial amyloidotic polyneuropathy after proteolysis by neutrophil serine elastases

Search Criteria
  • Amyloid
  • Cardiomyopathy