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Unusual duplication mutation in a surface loop of human transthyretin leads to an aggressive drug-resistant amyloid disease.

Klimtchuk ES, Prokaeva T, Frame NM, Abdullahi HA, Spencer B, Dasari S, Cui H, Berk JL, Kurtin PJ, Connors LH, Gursky O.

Proc Natl Acad Sci U S A. 2018 Jul 10;115(28):E6428-E6436. doi: 10.1073/pnas.1802977115. Epub 2018 Jun 25.


Features of atrial fibrillation in wild-type transthyretin cardiac amyloidosis: a systematic review and clinical experience.

Mints YY, Doros G, Berk JL, Connors LH, Ruberg FL.

ESC Heart Fail. 2018 Jun 19. doi: 10.1002/ehf2.12308. [Epub ahead of print]


Use of Serum Transthyretin as a Prognostic Indicator and Predictor of Outcome in Cardiac Amyloid Disease Associated With Wild-Type Transthyretin.

Hanson JLS, Arvanitis M, Koch CM, Berk JL, Ruberg FL, Prokaeva T, Connors LH.

Circ Heart Fail. 2018 Feb;11(2):e004000. doi: 10.1161/CIRCHEARTFAILURE.117.004000.


Monoclonal gammopathy of undetermined significance in systemic transthyretin amyloidosis (ATTR).

Phull P, Sanchorawala V, Connors LH, Doros G, Ruberg FL, Berk JL, Sarosiek S.

Amyloid. 2018 Mar;25(1):62-67. doi: 10.1080/13506129.2018.1436048. Epub 2018 Feb 9.


Oxidative Post-Translational Modifications of an Amyloidogenic Immunoglobulin Light Chain Protein.

Lu Y, Jiang Y, Prokaeva T, Connors LH, Costello CE.

Int J Mass Spectrom. 2017 May;416:71-79. doi: 10.1016/j.ijms.2016.11.006. Epub 2016 Nov 8.


Hereditary Renal Amyloidosis Associated With a Novel Apolipoprotein A-II Variant.

Prokaeva T, Akar H, Spencer B, Havasi A, Cui H, O'Hara CJ, Gursky O, Leszyk J, Steffen M, Browning S, Rosenberg A, Connors LH.

Kidney Int Rep. 2017 Jul 29;2(6):1223-1232. doi: 10.1016/j.ekir.2017.07.009. eCollection 2017 Nov. No abstract available.


Prevalence of mutant ATTR cardiac amyloidosis in elderly African Americans with heart failure.

Arvanitis M, Chan GG, Jacobson DR, Berk JL, Connors LH, Ruberg FL.

Amyloid. 2017 Dec;24(4):253-255. doi: 10.1080/13506129.2017.1391086. Epub 2017 Oct 20. No abstract available.


Serum Proteomic Variability Associated with Clinical Phenotype in Familial Transthyretin Amyloidosis (ATTRm).

Chan GG, Koch CM, Connors LH.

J Proteome Res. 2017 Nov 3;16(11):4104-4112. doi: 10.1021/acs.jproteome.7b00479. Epub 2017 Sep 27.


In vitro co-expression of human amyloidogenic immunoglobulin light and heavy chain proteins: a relevant cell-based model of AL amyloidosis.

Klimtchuk ES, Prokaeva TB, Spencer BH, Gursky O, Connors LH.

Amyloid. 2017 Jun;24(2):115-122. doi: 10.1080/13506129.2017.1336996. Epub 2017 Jun 20.


Retinol binding protein 4 (RBP4) concentration identifies V122I transthyretin cardiac amyloidosis.

Arvanitis M, Simon S, Chan G, Fine D, Beardsley P, LaValley M, Jacobson D, Koch C, Berk JL, Connors LH, Ruberg FL.

Amyloid. 2017 Mar;24(sup1):120-121. doi: 10.1080/13506129.2017.1295371. No abstract available.


Familial wild-type transthyretin cardiomyopathy.

Benson MD, Berk JL, Connors LH, Dasgupta NR.

Amyloid. 2017 Mar;24(sup1):91-92. doi: 10.1080/13506129.2017.1281118. No abstract available.


Blood Proteomic Profiling in Inherited (ATTRm) and Acquired (ATTRwt) Forms of Transthyretin-Associated Cardiac Amyloidosis.

Chan GG, Koch CM, Connors LH.

J Proteome Res. 2017 Apr 7;16(4):1659-1668. doi: 10.1021/acs.jproteome.6b00998. Epub 2017 Feb 23.


Identification of Transthyretin Cardiac Amyloidosis Using Serum Retinol-Binding Protein 4 and a Clinical Prediction Model.

Arvanitis M, Koch CM, Chan GG, Torres-Arancivia C, LaValley MP, Jacobson DR, Berk JL, Connors LH, Ruberg FL.

JAMA Cardiol. 2017 Mar 1;2(3):305-313. doi: 10.1001/jamacardio.2016.5864.


Immunoglobulin heavy light chain test quantifies clonal disease in patients with AL amyloidosis and normal serum free light chain ratio.

Prokaeva T, Spencer B, Sun F, O'Hara RM, Seldin DC, Connors LH, Sanchorawala V.

Amyloid. 2016 Dec;23(4):214-220. Epub 2016 Sep 28.


Heart Failure Resulting From Age-Related Cardiac Amyloid Disease Associated With Wild-Type Transthyretin: A Prospective, Observational Cohort Study.

Connors LH, Sam F, Skinner M, Salinaro F, Sun F, Ruberg FL, Berk JL, Seldin DC.

Circulation. 2016 Jan 19;133(3):282-90. doi: 10.1161/CIRCULATIONAHA.115.018852. Epub 2015 Dec 11.


Vertebral compression fractures as the initial presentation of AL amyloidosis: case series and review of literature.

Sarosiek S, Seldin DC, Connors LH, Spencer B, Murakami A, O'Hara C, Sanchorawala V.

Amyloid. 2015;22(3):156-62. doi: 10.3109/13506129.2015.1036983. Epub 2015 Jun 25.


Lysosomal dysfunction and impaired autophagy underlie the pathogenesis of amyloidogenic light chain-mediated cardiotoxicity.

Guan J, Mishra S, Qiu Y, Shi J, Trudeau K, Las G, Liesa M, Shirihai OS, Connors LH, Seldin DC, Falk RH, MacRae CA, Liao R.

EMBO Mol Med. 2015 May;7(5):688. doi: 10.15252/emmm.201505318. No abstract available.


Cooperative stabilization of transthyretin by clusterin and diflunisal.

Greene MJ, Klimtchuk ES, Seldin DC, Berk JL, Connors LH.

Biochemistry. 2015 Jan 20;54(2):268-78. doi: 10.1021/bi5011249. Epub 2014 Dec 24.


Genetic variation of the transthyretin gene in wild-type transthyretin amyloidosis (ATTRwt).

Sikora JL, Logue MW, Chan GG, Spencer BH, Prokaeva TB, Baldwin CT, Seldin DC, Connors LH.

Hum Genet. 2015 Jan;134(1):111-21. doi: 10.1007/s00439-014-1499-0. Epub 2014 Nov 4.


Lysosomal dysfunction and impaired autophagy underlie the pathogenesis of amyloidogenic light chain-mediated cardiotoxicity.

Guan J, Mishra S, Qiu Y, Shi J, Trudeau K, Las G, Liesa M, Shirihai OS, Connors LH, Seldin DC, Falk RH, MacRae CA, Liao R.

EMBO Mol Med. 2014 Nov;6(11):1493-507. doi: 10.15252/emmm.201404190. Erratum in: EMBO Mol Med. 2015 May;7(5):688.


Induced pluripotent stem cell modeling of multisystemic, hereditary transthyretin amyloidosis.

Leung A, Nah SK, Reid W, Ebata A, Koch CM, Monti S, Genereux JC, Wiseman RL, Wolozin B, Connors LH, Berk JL, Seldin DC, Mostoslavsky G, Kotton DN, Murphy GJ.

Stem Cell Reports. 2013 Oct 31;1(5):451-63. doi: 10.1016/j.stemcr.2013.10.003. eCollection 2013.


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.

Reddi HV, Jenkins S, Theis J, Thomas BC, Connors LH, Van Rhee F, Highsmith WE Jr.

J Mol Diagn. 2014 Jan;16(1):68-74. doi: 10.1016/j.jmoldx.2013.08.001. Epub 2013 Oct 30.


Transthyretin aggregate-specific antibodies recognize cryptic epitopes on patient-derived amyloid fibrils.

Phay M, Blinder V, Macy S, Greene MJ, Wooliver DC, Liu W, Planas A, Walsh DM, Connors LH, Primmer SR, Planque SA, Paul S, O'Nuallain B.

Rejuvenation Res. 2014 Apr;17(2):97-104. doi: 10.1089/rej.2013.1524.


Stanniocalcin1 is a key mediator of amyloidogenic light chain induced cardiotoxicity.

Guan J, Mishra S, Shi J, Plovie E, Qiu Y, Cao X, Gianni D, Jiang B, Del Monte F, Connors LH, Seldin DC, Lavatelli F, Rognoni P, Palladini G, Merlini G, Falk RH, Semigran MJ, Dec GW Jr, Macrae CA, Liao R.

Basic Res Cardiol. 2013 Sep;108(5):378. doi: 10.1007/s00395-013-0378-5. Epub 2013 Aug 28.


Human amyloidogenic light chain proteins result in cardiac dysfunction, cell death, and early mortality in zebrafish.

Mishra S, Guan J, Plovie E, Seldin DC, Connors LH, Merlini G, Falk RH, MacRae CA, Liao R.

Am J Physiol Heart Circ Physiol. 2013 Jul 1;305(1):H95-103. doi: 10.1152/ajpheart.00186.2013. Epub 2013 Apr 26.


Circulating matrix metalloproteinases and tissue inhibitors of metalloproteinases in cardiac amyloidosis.

Tanaka K, Essick EE, Doros G, Tanriverdi K, Connors LH, Seldin DC, Sam F.

J Am Heart Assoc. 2013 Mar 12;2(2):e005868. doi: 10.1161/JAHA.112.005868.


A new lysozyme tyr54asn mutation causing amyloidosis in a family of Swedish ancestry with gastrointestinal symptoms.

Girnius S, Skinner M, Spencer B, Prokaeva T, Bartholomew C, O'Hara C, Seldin DC, Connors LH.

Amyloid. 2012 Dec;19(4):182-5. doi: 10.3109/13506129.2012.723074. Epub 2012 Sep 14.


Detection of high-molecular-weight amyloid serum protein complexes using biological on-line tracer sedimentation.

Kingsbury JS, Laue TM, Chase SF, Connors LH.

Anal Biochem. 2012 Jun 15;425(2):151-6. doi: 10.1016/j.ab.2012.03.016. Epub 2012 Mar 27.


Doxycycline reduces fibril formation in a transgenic mouse model of AL amyloidosis.

Ward JE, Ren R, Toraldo G, Soohoo P, Guan J, O'Hara C, Jasuja R, Trinkaus-Randall V, Liao R, Connors LH, Seldin DC.

Blood. 2011 Dec 15;118(25):6610-7. doi: 10.1182/blood-2011-04-351643. Epub 2011 Oct 12.


Clinical features and survival in senile systemic amyloidosis: comparison to familial transthyretin cardiomyopathy.

Connors LH, Doros G, Sam F, Badiee A, Seldin DC, Skinner M.

Amyloid. 2011 Jun;18 Suppl 1:157-9. doi: 10.3109/13506129.2011.574354059. No abstract available. Erratum in: Amyloid. 2011 Jun;18 Suppl 1:159.


Metabolic phenotype in an AL amyloidosis transgenic mouse model.

Ward JE, SooHoo P, Toraldo G, Jasuja R, Connors LH, O'Hara C, Seldin DC.

Amyloid. 2011 Jun;18 Suppl 1:40-1. doi: 10.3109/13506129.2011.574354014. No abstract available. Erratum in: Amyloid. 2011 Jun;18 Suppl 1:41.


Dysregulation of miRNAs in AL amyloidosis.

Weng L, Spencer BH, SoohHoo PT, Connors LH, O'Hara CJ, Seldin DC.

Amyloid. 2011 Sep;18(3):128-35. doi: 10.3109/13506129.2011.588977. Epub 2011 Aug 11.


Preclinical development of siRNA therapeutics for AL amyloidosis.

Hovey BM, Ward JE, Soo Hoo P, O'Hara CJ, Connors LH, Seldin DC.

Gene Ther. 2011 Dec;18(12):1150-6. doi: 10.1038/gt.2011.69. Epub 2011 May 12.


Evidence for a functional role of the molecular chaperone clusterin in amyloidotic cardiomyopathy.

Greene MJ, Sam F, Soo Hoo PT, Patel RS, Seldin DC, Connors LH.

Am J Pathol. 2011 Jan;178(1):61-8. doi: 10.1016/j.ajpath.2010.11.015. Epub 2010 Dec 23.


The critical role of the constant region in thermal stability and aggregation of amyloidogenic immunoglobulin light chain.

Klimtchuk ES, Gursky O, Patel RS, Laporte KL, Connors LH, Skinner M, Seldin DC.

Biochemistry. 2010 Nov 16;49(45):9848-57. doi: 10.1021/bi101351c. Epub 2010 Oct 20.


Role of glycosaminoglycan sulfation in the formation of immunoglobulin light chain amyloid oligomers and fibrils.

Ren R, Hong Z, Gong H, Laporte K, Skinner M, Seldin DC, Costello CE, Connors LH, Trinkaus-Randall V.

J Biol Chem. 2010 Nov 26;285(48):37672-82. doi: 10.1074/jbc.M110.149575. Epub 2010 Sep 24.


Amyloidogenic light chains induce cardiomyocyte contractile dysfunction and apoptosis via a non-canonical p38alpha MAPK pathway.

Shi J, Guan J, Jiang B, Brenner DA, Del Monte F, Ward JE, Connors LH, Sawyer DB, Semigran MJ, Macgillivray TE, Seldin DC, Falk R, Liao R.

Proc Natl Acad Sci U S A. 2010 Mar 2;107(9):4188-93. doi: 10.1073/pnas.0912263107. Epub 2010 Feb 11.


Matrix metalloproteinases and their tissue inhibitors in cardiac amyloidosis: relationship to structural, functional myocardial changes and to light chain amyloid deposition.

Biolo A, Ramamurthy S, Connors LH, O'Hara CJ, Meier-Ewert HK, Soo Hoo PT, Sawyer DB, Seldin DC, Sam F.

Circ Heart Fail. 2008 Nov;1(4):249-57. doi: 10.1161/CIRCHEARTFAILURE.108.788687. Epub 2008 Oct 14. Erratum in: Circ Heart Fail. 2009 May;2(3):e3. Seldin, David S [corrected to Seldin, David C].


Cardiac amyloidosis in African Americans: comparison of clinical and laboratory features of transthyretin V122I amyloidosis and immunoglobulin light chain amyloidosis.

Connors LH, Prokaeva T, Lim A, Théberge R, Falk RH, Doros G, Berg A, Costello CE, O'Hara C, Seldin DC, Skinner M.

Am Heart J. 2009 Oct;158(4):607-14. doi: 10.1016/j.ahj.2009.08.006.


AL-Base: a visual platform analysis tool for the study of amyloidogenic immunoglobulin light chain sequences.

Bodi K, Prokaeva T, Spencer B, Eberhard M, Connors LH, Seldin DC.

Amyloid. 2009 Mar;16(1):1-8. doi: 10.1080/13506120802676781.


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.

Tam M, Seldin DC, Forbes BM, Connors LH, Skinner M, Oran B, Quillen K, Sanchorawala V.

Amyloid. 2009;16(2):103-7. doi: 10.1080/13506120902879574.


Serum transthyretin levels in senile systemic amyloidosis: effects of age, gender and ethnicity.

Buxbaum J, Koziol J, Connors LH.

Amyloid. 2008 Dec;15(4):255-61. doi: 10.1080/13506120802525285.


Amyloidogenic and associated proteins in systemic amyloidosis proteome of adipose tissue.

Lavatelli F, Perlman DH, Spencer B, Prokaeva T, McComb ME, Théberge R, Connors LH, Bellotti V, Seldin DC, Merlini G, Skinner M, Costello CE.

Mol Cell Proteomics. 2008 Aug;7(8):1570-83. doi: 10.1074/mcp.M700545-MCP200. Epub 2008 May 12.


The modulation of transthyretin tetramer stability by cysteine 10 adducts and the drug diflunisal. Direct analysis by fluorescence-detected analytical ultracentrifugation.

Kingsbury JS, Laue TM, Klimtchuk ES, Théberge R, Costello CE, Connors LH.

J Biol Chem. 2008 May 2;283(18):11887-96. doi: 10.1074/jbc.M709638200. Epub 2008 Mar 6.


Heterogeneity in primary structure, post-translational modifications, and germline gene usage of nine full-length amyloidogenic kappa1 immunoglobulin light chains.

Connors LH, Jiang Y, Budnik M, Théberge R, Prokaeva T, Bodi KL, Seldin DC, Costello CE, Skinner M.

Biochemistry. 2007 Dec 11;46(49):14259-71. Epub 2007 Nov 16.


Soft tissue, joint, and bone manifestations of AL amyloidosis: clinical presentation, molecular features, and survival.

Prokaeva T, Spencer B, Kaut M, Ozonoff A, Doros G, Connors LH, Skinner M, Seldin DC.

Arthritis Rheum. 2007 Nov;56(11):3858-68.


A new transthyretin variant (Glu61Gly) associated with cardiomyopathy.

Rosenzweig M, Skinner M, Prokaeva T, Théberge R, Costello C, Drachman BM, Connors LH.

Amyloid. 2007 Mar;14(1):65-71.


Expression, purification, and in vitro cysteine-10 modification of native sequence recombinant human transthyretin.

Kingsbury JS, Klimtchuk ES, Théberge R, Costello CE, Connors LH.

Protein Expr Purif. 2007 Jun;53(2):370-7. Epub 2007 Jan 17.


Detailed structural analysis of amyloidogenic wild-type transthyretin using a novel purification strategy and mass spectrometry.

Kingsbury JS, Théberge R, Karbassi JA, Lim A, Costello CE, Connors LH.

Anal Chem. 2007 Mar 1;79(5):1990-8. Epub 2007 Jan 30.


Quantitative serum free light chain assay in the diagnostic evaluation of AL amyloidosis.

Akar H, Seldin DC, Magnani B, O'Hara C, Berk JL, Schoonmaker C, Cabral H, Dember LM, Sanchorawala V, Connors LH, Falk RH, Skinner M.

Amyloid. 2005 Dec;12(4):210-5.


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