Harry C Dietz

Harry C Dietz

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Harry C Dietz

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Management of the aortic arch in patients with Loeys-Dietz syndrome.

J Thorac Cardiovasc Surg 2019 Sep 9. Epub 2019 Sep 9.

Division of Cardiac Surgery, Johns Hopkins Medical Institutions, Baltimore, Md. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2019.07.130DOI Listing
September 2019

Regenerative and durable small-diameter graft as an arterial conduit.

Proc Natl Acad Sci U S A 2019 Jun 10;116(26):12710-12719. Epub 2019 Jun 10.

Department of Biomedical Engineering, Johns Hopkins University School of Medicine, Baltimore, MD 21205;

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http://dx.doi.org/10.1073/pnas.1905966116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6601275PMC
June 2019

Proteomics reveals Rictor as a noncanonical TGF-β signaling target during aneurysm progression in Marfan mice.

Am J Physiol Heart Circ Physiol 2018 11 13;315(5):H1112-H1126. Epub 2018 Jul 13.

Advanced Clinical Biosystems Research Institute, The Smidt Heart Institute, Cedars-Sinai Medical Center , Los Angeles, California.

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http://dx.doi.org/10.1152/ajpheart.00089.2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335018PMC
November 2018

Pregnancy after Aortic Root Replacement in Marfan's Syndrome: A Case Series and Review of the Literature.

AJP Rep 2018 Oct 18;8(4):e234-e240. Epub 2018 Oct 18.

Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, Missouri.

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http://dx.doi.org/10.1055/s-0038-1675347DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6193806PMC
October 2018

Spondylolisthesis is Common, Early, and Severe in Loeys-Dietz Syndrome.

J Pediatr Orthop 2018 Sep;38(8):e455-e461

Departments of Pediatrics.

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http://dx.doi.org/10.1097/BPO.0000000000001203DOI Listing
September 2018

Carotid Artery Tortuosity Index Is Associated With the Need for Early Aortic Root Replacement in Patients With Loeys-Dietz Syndrome.

J Comput Assist Tomogr 2018 Sep/Oct;42(5):747-753

From the The Russell H. Morgan Department of Radiology and Radiological Science.

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http://dx.doi.org/10.1097/RCT.0000000000000764DOI Listing
September 2018

First evidence of maternally inherited mosaicism in TGFBR1 and subtle primary myocardial changes in Loeys-Dietz syndrome: a case report.

BMC Med Genet 2018 09 15;19(1):170. Epub 2018 Sep 15.

Mechanical Assistance Device and Artificial Heart Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

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https://bmcmedgenet.biomedcentral.com/articles/10.1186/s1288
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http://dx.doi.org/10.1186/s12881-018-0661-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6139163PMC
September 2018

Management of Scoliosis in Patients With Loeys-Dietz Syndrome.

J Pediatr Orthop 2017 Dec;37(8):e492-e499

*The Johns Hopkins University School of Medicine †The Institute of Genetic Medicine Departments of ‡Pediatrics §Pediatric Orthopaedic Surgery, The Johns Hopkins University, Baltimore, MD.

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http://dx.doi.org/10.1097/BPO.0000000000000833DOI Listing
December 2017

TGF-β Family Signaling in Connective Tissue and Skeletal Diseases.

Cold Spring Harb Perspect Biol 2017 Nov 1;9(11). Epub 2017 Nov 1.

Department of Orthopedic Surgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104.

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http://cshperspectives.cshlp.org/lookup/doi/10.1101/cshpersp
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http://dx.doi.org/10.1101/cshperspect.a022269DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5666637PMC
November 2017

Moderately Elevated Homocysteine Does Not Contribute to Thoracic Aortic Aneurysm in Mice.

J Nutr 2017 07 24;147(7):1290-1295. Epub 2017 May 24.

National Human Genome Research Institute, NIH, Bethesda, MD;

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http://dx.doi.org/10.3945/jn.117.251173DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5483969PMC
July 2017

Aortic Root Replacement for Children With Loeys-Dietz Syndrome.

Ann Thorac Surg 2017 May 31;103(5):1513-1518. Epub 2017 Mar 31.

Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S00034975173010
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http://dx.doi.org/10.1016/j.athoracsur.2017.01.053DOI Listing
May 2017

2016 Presidential Address: Let's Make Human Genetics Great (Again): The Importance of Beauty in Science.

Authors:
Harry C Dietz

Am J Hum Genet 2017 03;100(3):379-384

Howard Hughes Medical Institute; Institute of Genetic Medicine, Departments of Pediatrics and Medicine, Johns Hopkins University School of Medicine, Miller Research Building, Room 539, 733 North Broadway, Baltimore, MD 21205, USA. Electronic address:

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http://dx.doi.org/10.1016/j.ajhg.2017.01.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5339082PMC
March 2017

Cardiovascular operations for Loeys-Dietz syndrome: Intermediate-term results.

J Thorac Cardiovasc Surg 2017 02 16;153(2):406-412. Epub 2016 Nov 16.

Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2016.10.088DOI Listing
February 2017

Simplified mitral valve repair in pediatric patients with connective tissue disorders.

J Thorac Cardiovasc Surg 2017 02 23;153(2):399-403. Epub 2016 Sep 23.

Division of Cardiac Surgery, Johns Hopkins University School of Medicine, Baltimore, Md.

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http://dx.doi.org/10.1016/j.jtcvs.2016.09.039DOI Listing
February 2017

Quantifying Health Status and Function in Marfan Syndrome.

J Surg Orthop Adv 2016 ;25(1):34-40

Department of Orthopaedic Surgery, The Johns Hopkins University, Baltimore, Maryland.

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July 2016

Massive hemoptysis in Loeys-Dietz syndrome.

Am J Med Genet A 2016 Mar 27;170(3):725-7. Epub 2015 Nov 27.

The Howard Hughes Medical Institute, Baltimore, Maryland.

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http://dx.doi.org/10.1002/ajmg.a.37487DOI Listing
March 2016

Long-term outcomes of aortic root operations for Marfan syndrome: A comparison of Bentall versus aortic valve-sparing procedures.

J Thorac Cardiovasc Surg 2016 Feb 27;151(2):330-6. Epub 2015 Oct 27.

Division of Cardiac Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md.

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http://dx.doi.org/10.1016/j.jtcvs.2015.10.068DOI Listing
February 2016

Dysregulated TGF-β signaling alters bone microstructure in a mouse model of Loeys-Dietz syndrome.

J Orthop Res 2015 Oct 14;33(10):1447-54. Epub 2015 Jul 14.

Department of Orthopaedic Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland.

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http://dx.doi.org/10.1002/jor.22920DOI Listing
October 2015

Performant Mutation Identification Using Targeted Next-Generation Sequencing of 14 Thoracic Aortic Aneurysm Genes.

Hum Mutat 2015 Aug 13;36(8):808-14. Epub 2015 Jun 13.

Department of Medical Genetics, Faculty of Medicine and Health Sciences, University of Antwerp and Antwerp University Hospital, Antwerp, Belgium.

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http://dx.doi.org/10.1002/humu.22802DOI Listing
August 2015

Determinants of Aortic Root Dilatation and Reference Values Among Young Adults Over a 20-Year Period: Coronary Artery Risk Development in Young Adults Study.

Hypertension 2015 Jul 4;66(1):23-9. Epub 2015 May 4.

From the Department of Medicine and Medical Genetics, Johns Hopkins University, Baltimore, MD (G.T.-T., A.L.C.A., E.-Y.C., O.G., H.C.D., J.A.C.L.); Department of Cardiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain (G.T.-T., D.G.-D., A.E.); Department of Epidemiology, University of Minnesota, Minneapolis (D.R.J.); Department of Preventive Medicine, Northwestern University, Chicago, IL (K.L.); Kaiser Permanente, Division of Research, Oakland, CA (S.S.); Division of Preventive Medicine, University of Alabama, Birmingham (C.E.L.); and Division of Cardiology, Nemours Cardiac Center, A. I. DuPont Hospital for Children, Wilmington, DE (S.G.).

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http://dx.doi.org/10.1161/HYPERTENSIONAHA.115.05156DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4465858PMC
July 2015

One integrin to rule them all?

Authors:
Harry C Dietz

Sci Transl Med 2015 May;7(288):288fs21

Institute of Genetic Medicine, Howard Hughes Medical Institute, and Smilow Center for Marfan Syndrome Research, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.

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http://stm.sciencemag.org/content/scitransmed/7/288/288fs21.
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http://stm.sciencemag.org/cgi/doi/10.1126/scitranslmed.aab00
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http://dx.doi.org/10.1126/scitranslmed.aab0044DOI Listing
May 2015

Vascular smooth muscle cell phenotypic changes in patients with Marfan syndrome.

Arterioscler Thromb Vasc Biol 2015 Apr 15;35(4):960-72. Epub 2015 Jan 15.

From the Department of Cell Biology, Immunology and Neurosciences (E.C.-M., T.M., C.S.-P, J.S., D.G, Y.M., V.H., E.S., G.E.), Departments of Physiological Sciences I (J.J.U., D.N.) and Physiological Sciences II (I.F.), Department of Pathology and Experimental Therapeutics (E.C.), University of Barcelona School of Medicine, Barcelona, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain (M.C., G.E.); Institut de Nanociència i Nanotecnologia (IN2UB), Barcelona, Spain (G.E.); Institut de Bioenginyeria de Catalunya (IBEC), Barcelona, Spain and CIBER de Enfermedades Respiratorias (CIBERES) (D.N.); Vascular Biology Laboratory, Heart Institute (InCor), University of São Paulo School of Medicine, São Paulo, Brazil (T.M.); Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), L'Hospitalet de Llobregat, Barcelona, Spain (E.C.-M., J.L.-L. L.C., E.B., I.F.); Centro de Biología Molecular Severo Ochoa (CSIC-UAM), Madrid, Spain (O.B., F.R.-P.); Hospital de Bellvitge-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain (C.G.-C., E.C., D.T.); Cardiovascular Surgery Department, Hospital Clínic i Provincial, Barcelona, Spain (M.C.); Cardiac Surgery Department, Marfan Syndrome Unit, Hospital Universitario 12 de Octubre, Madrid, Spain (A.F.); and William S. Smilow Center for Marfan Syndrome Research, Howard Hughes Medical Institute, Johns Hopkins University School of Medicine, Baltimore, MD (H.C.D.).

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http://dx.doi.org/10.1161/ATVBAHA.114.304412DOI Listing
April 2015

Potential Phenotype-Genotype Correlation in Marfan Syndrome: When Less is More?

Authors:
Harry C Dietz

Circ Cardiovasc Genet 2015 Apr;8(2):256-60

From the Departments of Pediatrics, Medicine and Molecular Biology and Genetics, Howard Hughes Medical Institute, William S. Smilow Center for Marfan Syndrome Research, Johns Hopkins University School of Medicine, Baltimore, MD.

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http://dx.doi.org/10.1161/CIRCGENETICS.115.001040DOI Listing
April 2015

Atenolol versus Losartan in Marfan's Syndrome.

N Engl J Med 2015 03;372(10):980-1

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http://dx.doi.org/10.1056/NEJMc1500128DOI Listing
March 2015

High prevalence of cervical deformity and instability requires surveillance in Loeys-Dietz syndrome.

J Bone Joint Surg Am 2015 Mar;97(5):411-9

c/o Rachel Box, MS, ELS, Senior Editor and Director, Editorial Services, Department of Orthopaedic Surgery, The Johns Hopkins University/Johns Hopkins Bayview Medical Center, 4940 Eastern Avenue, #A665, Baltimore, MD 21224-2780. E-mail address for R. Box:

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http://dx.doi.org/10.2106/JBJS.N.00680DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4344594PMC
March 2015

Mutations in SGOL1 cause a novel cohesinopathy affecting heart and gut rhythm.

Nat Genet 2014 Nov 5;46(11):1245-9. Epub 2014 Oct 5.

1] Cardiovascular Genetics, Department of Pediatrics, Centre Hospitalier Universitaire Sainte-Justine Research Centre, Université de Montréal, Montreal, Quebec, Canada. [2] Department of Pediatrics, Université de Montréal, Montreal, Quebec, Canada. [3] Department of Biochemistry, Université de Montréal, Montreal, Quebec, Canada.

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http://dx.doi.org/10.1038/ng.3113DOI Listing
November 2014

Atenolol versus losartan in children and young adults with Marfan's syndrome.

N Engl J Med 2014 Nov 18;371(22):2061-71. Epub 2014 Nov 18.

From Boston Children's Hospital, Boston (R.V.L., S.D.C., E.S.S.T., J.C.L.); Johns Hopkins University School of Medicine, Baltimore (H.C.D.); New England Research Institutes, Watertown, MA (L.A.S., S.D.C., S.C., G.L.K.); Primary Children's Hospital and the University of Utah, Salt Lake City (A.T.Y.); Hospital for Sick Children, Toronto (T.J.B., E.R.); National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD (G.D.P., V.L.P., M.P.S.); Medical University of South Carolina, Charleston (A.M.A.); Cincinnati Children's Medical Center, Cincinnati (D.W.B.); Washington University School of Medicine, St. Louis (A.C.B., A.M.S.); Ghent University Hospital, Ghent, Belgium (J.D.B., B.L.L.); Icahn School of Medicine at Mount Sinai (B.D.G.), Children's Hospital of New York (W.W.L.), and Weill Medical College of Cornell University (M.J.R.) - all in New York; Rady Children's Hospital, University of California, San Diego (P.D.G.); Texas Children's Hospital, Houston (A.L.); Vanderbilt University School of Medicine, Nashville (L.W.M.); Seattle Children's Hospital, Seattle (A.K.O.); Children's Hospital of Philadelphia (S.M.P.) and the University of Pennsylvania (R.E.P.), Philadelphia; Children's Hospital and Clinics of Minnesota, Minneapolis (M.E.P.); Duke University Medical Center, Durham, NC (S.B.W.); Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago (L.T.Y.); and the University of Texas Southwestern Medical Center, Dallas (L.M.).

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http://dx.doi.org/10.1056/NEJMoa1404731DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4386623PMC
November 2014

Histone deacetylase inhibition rescues structural and functional brain deficits in a mouse model of Kabuki syndrome.

Sci Transl Med 2014 Oct;6(256):256ra135

McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. Division of Pediatric Cardiology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. Howard Hughes Medical Institute, Baltimore, MD 21205, USA.

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http://dx.doi.org/10.1126/scitranslmed.3009278DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4406328PMC
October 2014

The genetic basis of aortic aneurysm.

Cold Spring Harb Perspect Med 2014 Sep 2;4(9):a015909. Epub 2014 Sep 2.

McKusick-Nathans Institute of Genetic Medicine, Departments of Pediatrics, Medicine, and Molecular Biology & Genetics, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205 Howard Hughes Medical Institute, Baltimore, Maryland 21205.

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http://dx.doi.org/10.1101/cshperspect.a015909DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4143103PMC
September 2014

Loeys-Dietz syndrome: a primer for diagnosis and management.

Genet Med 2014 Aug 27;16(8):576-87. Epub 2014 Feb 27.

1] McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA [2] Howard Hughes Institute, Chevy Chase, Maryland, USA.

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http://dx.doi.org/10.1038/gim.2014.11DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4131122PMC
August 2014

Response to Pyeritz et al.

Genet Med 2014 Aug;16(8):642-4

1] Institute of Genetic Medicine, Johns Hopkins University, Baltimore, Maryland, USA [2] Howard Hughes Institute, Chevy Chase, Maryland, USA.

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http://dx.doi.org/10.1038/gim.2014.59DOI Listing
August 2014

CT angiographic evaluation of genetic vascular disease: role in detection, staging, and management of complex vascular pathologic conditions.

AJR Am J Roentgenol 2014 May;202(5):1120-9

1 The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins Hospital, 601 N Caroline St, Baltimore, MD 21287.

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http://dx.doi.org/10.2214/AJR.13.11485DOI Listing
May 2014

Mandibuloacral Dysplasia Caused by LMNA Mutations and Uniparental Disomy.

Case Rep Genet 2014 3;2014:508231. Epub 2014 Feb 3.

University of Chicago, 5841 South Maryland Avenue, Chicago, IL 60637, USA ; Department of Human Genetics, University of Chicago, 5841 South Maryland Avenue, Chicago, IL 60637, USA.

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http://dx.doi.org/10.1155/2014/508231DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3930135PMC
March 2014

Integrin-modulating therapy prevents fibrosis and autoimmunity in mouse models of scleroderma.

Nature 2013 Nov 9;503(7474):126-30. Epub 2013 Oct 9.

McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.

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http://dx.doi.org/10.1038/nature12614DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3992987PMC
November 2013

Mitral valve disease in patients with Marfan syndrome undergoing aortic root replacement.

Circulation 2013 Sep;128(11 Suppl 1):S243-7

Division of Cardiovascular Surgery (M.R.K., H.V.S., Z.L.) and Division of Cardiovascular Diseases (H.C.), Mayo Clinic, Rochester, MN; Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX (I.V., S.A.L., J.S.C.); Department of Cardiovascular Surgery, Texas Heart Institute, Houston, TX (I.V., S.A.L., J.S.C.); and McKusick-Nathans Institute of Genetic Medicine and Department of Pediatrics, Johns Hopkins University School of Medicine and Howard Hughes Medical Institute, Baltimore, MD (H.C.D.).

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http://dx.doi.org/10.1161/CIRCULATIONAHA.112.000113DOI Listing
September 2013

Increased fracture risk and low bone mineral density in patients with loeys-dietz syndrome.

Am J Med Genet A 2013 Aug 4;161A(8):1910-4. Epub 2013 Jul 4.

Department of Orthopaedic Surgery, The Johns Hopkins University, Baltimore, MD 21224-2780, USA.

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http://dx.doi.org/10.1002/ajmg.a.36029DOI Listing
August 2013

Genotype-phenotype correlation in patients with bicuspid aortic valve and aneurysm.

J Thorac Cardiovasc Surg 2013 Jul 23;146(1):158-165.e1. Epub 2012 Oct 23.

McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.

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http://dx.doi.org/10.1016/j.jtcvs.2012.09.060DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3697122PMC
July 2013

LMNA-associated cardiocutaneous progeria: an inherited autosomal dominant premature aging syndrome with late onset.

Am J Med Genet A 2013 Jul 10;161A(7):1599-611. Epub 2013 May 10.

Department of Cell Biology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.

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http://dx.doi.org/10.1002/ajmg.a.35971DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3740161PMC
July 2013

Inhibition of nonsense-mediated RNA decay activates autophagy.

Mol Cell Biol 2013 Jun 18;33(11):2128-35. Epub 2013 Mar 18.

Department of Biochemistry and Molecular Pharmacology, New York University School of Medicine, New York, NY,USA.

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http://mcb.asm.org/cgi/doi/10.1128/MCB.00174-13
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http://dx.doi.org/10.1128/MCB.00174-13DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3648072PMC
June 2013

Bone lessons from Marfan syndrome and related disorders: fibrillin, TGF-B and BMP at the balance of too long and too short.

Pediatr Endocrinol Rev 2013 Jun;10 Suppl 2:417-23

Center for Medical Genetics, University of Antwerp, Antwerp, BE.

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June 2013

Tetralogy of Fallot and aortic root dilation: a long-term outlook.

Pediatr Cardiol 2013 Apr 19;34(4):809-16. Epub 2012 Oct 19.

Division of Cardiology, Tufts Medical Center, 800 Washington Street, Boston, MA 02111, USA.

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http://dx.doi.org/10.1007/s00246-012-0537-8DOI Listing
April 2013

Targeting collagen strands by photo-triggered triple-helix hybridization.

Proc Natl Acad Sci U S A 2012 Sep 27;109(37):14767-72. Epub 2012 Aug 27.

Department of Chemistry, Johns Hopkins University, Baltimore, MD 21218, USA.

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http://dx.doi.org/10.1073/pnas.1209721109DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3443117PMC
September 2012

Matrix-dependent perturbation of TGFβ signaling and disease.

FEBS Lett 2012 Jul 26;586(14):2003-15. Epub 2012 May 26.

Howard Hughes Medical Institute, Chevy Chase, MD 20815, USA.

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http://dx.doi.org/10.1016/j.febslet.2012.05.027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3426037PMC
July 2012

Vascular complications of Ehlers-Danlos syndrome: CT findings.

AJR Am J Roentgenol 2012 Feb;198(2):482-7

The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins Hospital, 601 N Caroline St, JHOC 3254, Baltimore, MD 21287, USA.

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http://dx.doi.org/10.2214/AJR.11.6603DOI Listing
February 2012

Altered tissue behavior of a non-aneurysmal descending thoracic aorta in the mouse model of Marfan syndrome.

Cell Tissue Res 2012 Jan 22;347(1):267-77. Epub 2011 Nov 22.

Graduate Interdisciplinary Program of Biomedical Engineering, University of Arizona, Tucson, Ariz., USA.

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http://dx.doi.org/10.1007/s00441-011-1270-yDOI Listing
January 2012

Angiotensin receptor blockade attenuates cigarette smoke-induced lung injury and rescues lung architecture in mice.

J Clin Invest 2012 Jan 19;122(1):229-40. Epub 2011 Dec 19.

Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.

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http://dx.doi.org/10.1172/JCI46215DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3248282PMC
January 2012

Mitral valve disease in Marfan syndrome and related disorders.

J Cardiovasc Transl Res 2011 Dec 25;4(6):741-7. Epub 2011 Aug 25.

Division of Cardiology, Johns Hopkins University, Ross 1049 720 Rutland Avenue, Baltimore, MD 21205, USA.

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http://dx.doi.org/10.1007/s12265-011-9314-yDOI Listing
December 2011

Pravastatin reduces Marfan aortic dilation.

Circulation 2011 Sep;124(11 Suppl):S168-73

Department of Surgery, Royal College of Surgeons in Ireland, Dublin, Ireland.

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http://dx.doi.org/10.1161/CIRCULATIONAHA.110.012187DOI Listing
September 2011

Valve-sparing aortic root replacement in Loeys-Dietz syndrome.

Ann Thorac Surg 2011 Aug;92(2):556-60; discussion 560-1

Johns Hopkins Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.

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http://dx.doi.org/10.1016/j.athoracsur.2011.04.003DOI Listing
August 2011

Perturbation of thymocyte development in nonsense-mediated decay (NMD)-deficient mice.

Proc Natl Acad Sci U S A 2011 Jun 13;108(26):10638-43. Epub 2011 Jun 13.

Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.

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http://dx.doi.org/10.1073/pnas.1019352108DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3127929PMC
June 2011

Lessons on the pathogenesis of aneurysm from heritable conditions.

Nature 2011 May;473(7347):308-16

Division of Pediatric Cardiology, Department of Pediatrics, Johns Hopkins Medical Institutions, Baltimore, Maryland 21205-1832, USA.

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http://dx.doi.org/10.1038/nature10145DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3622871PMC
May 2011

Angiotensin II type 2 receptor signaling attenuates aortic aneurysm in mice through ERK antagonism.

Science 2011 Apr;332(6027):361-5

Howard Hughes Medical Institute and Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.

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http://dx.doi.org/10.1126/science.1192152DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3097422PMC
April 2011

Valve-sparing aortic root replacement in children: intermediate-term results.

Interact Cardiovasc Thorac Surg 2011 Mar 30;12(3):415-9, discussion 419. Epub 2010 Nov 30.

Johns Hopkins Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital, Baltimore, MD 21287, USA.

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http://dx.doi.org/10.1510/icvts.2010.255596DOI Listing
March 2011

Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome.

Hum Mol Genet 2010 Dec 24;19(24):4790-8. Epub 2010 Sep 24.

Department of Pharmacology and Systems Therapeutics at the Mount Sinai School of Medicine, New York, NY 10029, USA.

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http://dx.doi.org/10.1093/hmg/ddq409DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2989889PMC
December 2010

The diagnostic value of the facial features of Marfan syndrome.

J Child Orthop 2010 Dec 31;4(6):545-51. Epub 2010 Oct 31.

Department of Orthopaedic Surgery, Johns Hopkins Bayview Medical Center, 4940 Eastern Ave., #A665, Baltimore, MD 21224-2780 USA.

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http://dx.doi.org/10.1007/s11832-010-0295-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2981714PMC
December 2010

New therapeutic approaches to mendelian disorders.

Authors:
Harry C Dietz

N Engl J Med 2010 Aug;363(9):852-63

McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine, 733 N. Broadway St., Baltimore, MD 21205, USA.

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http://dx.doi.org/10.1056/NEJMra0907180DOI Listing
August 2010