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George A. Porter Jr, M.D., Ph.D.

George A. Porter Jr, M.D., Ph.D.

Pediatrics , Pediatric Cardiology

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UR Medicine Faculty The University of Rochester Medical Faculty Group (URMFG) consists of over 900 specialist and primary care providers spanning 19 departments. URMFG is certified by the National Committee for Quality Assurance.
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Contact

Appointment (585) 275-6108
Fax (585) 442-0104
Administrative (585) 275-6096

About Me

General Pediatric Cardiology

Pediatric Cardiology Research
General Pediatric Cardiology

Pediatric Cardiology Research

Certified Specialties

Pediatric Cardiology - American Board of Pediatrics

Faculty Appointments

Professor - Department of Pediatrics, Cardiology (SMD)

Rhea and Raymond White Professorship in Pediatric Cardiology - Department of Pediatrics (SMD)

Chief - Department of Pediatrics, Cardiology (SMD)

Professor - Department of Medicine, Aab Cardiovascular Research Institute (SMD) - Joint

Professor - Department of Pharmacology and Physiology (SMD) - Joint

Credentials

Residency & Fellowship

Fellowship, Pediatric Cardiology, Yale New Haven Hospital. 1997 - 2000

Residency, Pediatrics, Yale University School of Medicine. 1995 - 1997

Internship, Pediatrics, Yale New Haven Hospital. 1994 - 1995

Undergraduate, University of Notre Dame.

Education

MD | University of Maryland School of Medicine. 1994

PhD | University of Maryland School of Medicine. 1993

Awards

Mentor of the Year Award. 2018

Ruth A. Lawrence Academic Faculty Service Award in Research, UR Medicine, Department of Pediatrics. 2015

Second place, Basic Cardiovascular Science Poster Competition (Research Symposium). 2008

The Mae Gailani Junior Faculty Award for Uncompromising Dedication to Research and Patient Care. 2004

AHA Travel Award to the 1999 Weinstein Cardiovascular Development Conference. 1999

The American Federation for Clinical Research Medical Student Award. 1994

Dr. J. Edmund Bradley Award for Excellence in Pediatrics. 1994

Extended Neuroscience Research Award, The American Academy of Neurology. 1992

Research

Dr. Porter's laboratory studies mechanisms that control cardiac development, concentrating on the roles played by the intracellular organelles, mitochondria. Using in vivo and in vitro mouse models of cardiac development, the lab has shown that mitochondrial structure and function changes dramatical...
Dr. Porter's laboratory studies mechanisms that control cardiac development, concentrating on the roles played by the intracellular organelles, mitochondria. Using in vivo and in vitro mouse models of cardiac development, the lab has shown that mitochondrial structure and function changes dramatically in cardiac myocytes as the embryonic heart forms. In particular, we have found that closure of the mitochondrial permeability transition pore (PTP) between the early and mid-embryonic period leads to a maturation of the structure of individual mitochondria and of the mitochondrial network throughout the cell. This also leads to an activation of oxidative phosphorylation, or ATP production, by mitochondria as the heart develops. These changes also cause a drop in cellular oxidative stress due to altered mitochondrial production of reactive oxygen species, and this signals to the myocytes to undergo further differentiation. More recent research finds that the same phenomena occur in the neonatal heart, and that closure of the PTP in the neonate increases cardiac function in vivo.
These findings have led to additional studies. 1. Determining that the PTP is derived from ATP synthase the electron transport chain complex that makes ATP. 2. Determining the mechanisms that control the activity of the PTP. 3. Investigating the mechanisms by which mitochondria control oxidative stress in the embryonic heart. 5. Determining how mitochondria regulate differentiation of cardiac myocytes in the neonatal period and how oxygen levels regulate these changes and cause maturation of the infant heart.
Finally, Dr. Porter is the site principal investigator at the University of Rochester for the Pediatric Cardiac Genomics Consortium. This international, multicenter arm of the NIH Bench to Bassinet program (http://www.benchtobassinet.net/) has enrolled over 10,000 patients with congenital heart defects to perform genotype-phenotype correlation using advanced genetic testing. This data derived from this study is being used to discover new genes that cause human congenital heart defects and to test the pathogenesis of these genes in animal models through collaboration with the Cardiovascular Development Consortium of the Bench to Bassinet program. The most recent version of this study will determine the relationship between genetic mutations, heart defects, and neurodevelopmental outcomes.
These studies have been funded by the Charles H. Hood Foundation, the Children's Cardiomyopathy Foundation, Founder's Affiliate of American Heart Association, the NIH, Pfizer, the Strong Children's Research Center, and the University of Rochester Clinical and Translational Science Institute (CTSI), the Aab Cardiovascular Research Institute (CVRI), and the United Mitochondrial Disease Foundation.

Patents

Compositions and Methods for Enhancing Cardiac Function in the Neonate

Issue date: January 15, 2019

Patent #: 10,179,161

Country: United States

Inventors: George A Porter, Jr.

Publications

Journal Articles

ORE identifies extreme expression effects enriched for rare variants.

Richter F, Hoffman GE, Manheimer KB, Patel N, Sharp AJ, McKean D, Morton SU, DePalma S, Gorham J, Kitaygorodksy A, Porter GA, Giardini A, Shen Y, Chung WK, Seidman JG, Seidman CE, Schadt EE, Gelb BD

Bioinformatics.. 2019 October 1535 (20):3906-3912. Epub 1900 01 01.

Mitochondrial Oxidative Phosphorylation defect in the Heart of Subjects with Coronary Artery Disease.

Ait-Aissa K, Blaszak SC, Beutner G, Tsaih SW, Morgan G, Santos JH, Flister MJ, Joyce DL, Camara AKS, Gutterman DD, Donato AJ, Porter GA, Beyer AM

Scientific reports.. 2019 May 209 (1):7623. Epub 05/20/2019.

Dual role of inorganic polyphosphate in cardiac myocytes: The importance of polyP chain length for energy metabolism and mPTP activation.

Seidlmayer LK, Gomez-Garcia MR, Shiba T, Porter GA, Pavlov EV, Bers DM, Dedkova EN

Archives of biochemistry and biophysics.. 2019 February 15662 :177-189. Epub 12/17/2018.

Reply to 'Double-outlet right ventricle is not hypoplastic left heart syndrome'.

Lo CW, Liu X, Gabriel GC, Martin LJ, Porter GA, Benson DW

Nature genetics.. 2019 February 51 (2):198-199. Epub 1900 01 01.

GATA6 mutations in induced pluripotent stem cells inform developmental mechanisms for heart, pancreas, and diaphragm. eLIFE. Submitted for Publication.

Sharma A, Wasson L, Willcox JAL, Morton SU , Gorham JM, DeLaughter DM, Neyazi M, Manuel Schmid M, Agarwal R , Jang MY, Toepfer CN, Ward T, Kim Y, Pereira AC, DePalma SR, Tai A , Kim S, Conner D, Bernstein D, Gelb BC, Chung WK, Goldmuntz E, Porter G, Tristani-Firouzi M, Srivastava D, Seidman JG, Seidman CE.

2019; .

GATA6 mutations in induced pluripotent stem cells inform developmental mechanisms for heart, pancreas, and diaphragm. 2019. eLIFE. Submitted for Publication

Sharma A, Wasson L, Willcox JAL, Morton SU , Gorham JM, DeLaughter DM, Neyazi M, Manuel Schmid M, Agarwal R , Jang MY, Toepfer CN, Ward T, Kim Y, Pereira AC, DePalma SR, Tai A , Kim S, Conner D, Bernstein D, Gelb BC, Chung WK, Goldmuntz E, Porter G, Tristani-Firouzi M, Srivastava D, Seidman JG, Seidman CE.

2019; .

Potassium conservation is impaired in mice with reduced renal expression of Kir4.1.

Malik S, Lambert E, Zhang J, Wang T, Clark HL, Cypress M, Goldman BI, Porter GA, Pena S, Nino W, Gray DA

American journal of physiology. Renal physiology.. 2018 November 1315 (5):F1271-F1282. Epub 08/15/2018.

Metabolomics reveals critical adrenergic regulatory checkpoints in glycolysis and pentose-phosphate pathways in embryonic heart.

Peoples JNR, Maxmillian T, Le Q, Nadtochiy SM, Brookes PS, Porter GA, Davidson VL, Ebert SN

The Journal of biological chemistry.. 2018 May 4293 (18):6925-6941. Epub 03/14/2018.

The Congenital Heart Disease Genetic Network Study: Cohort description.

Hoang TT, Goldmuntz E, Roberts AE, Chung WK, Kline JK, Deanfield JE, Giardini A, Aleman A, Gelb BD, Mac Neal, Porter GA, Kim R, Brueckner M, Lifton RP, Edman S, Woyciechowski S, Mitchell LE, Agopian AJ

PloS one.. 2018 13 (1):e0191319. Epub 01/19/2018.

Cyclophilin D regulates the dynamic assembly of mitochondrial ATP synthase into synthasomes.

Beutner G, Alanzalon RE, Porter GA

Scientific reports.. 2017 November 37 (1):14488. Epub 11/03/2017.

Contribution of rare inherited and de novo variants in 2,871 congenital heart disease probands.

Jin SC, Homsy J, Zaidi S, Lu Q, Morton S, DePalma SR, Zeng X, Qi H, Chang W, Sierant MC, Hung WC, Haider S, Zhang J, Knight J, Bjornson RD, Castaldi C, Tikhonoa IR, Bilguvar K, Mane SM, Sanders SJ, Mital S, Russell MW, Gaynor JW, Deanfield J, Giardini A, Porter GA, Srivastava D, Lo CW, Shen Y, Watkins WS, Yandell M, Yost HJ, Tristani-Firouzi M, Newburger JW, Roberts AE, Kim R, Zhao H, Kaltman JR, Goldmuntz E, Chung WK, Seidman JG, Gelb BD, Seidman CE, Lifton RP, Brueckner M

Nature genetics.. 2017 November 49 (11):1593-1601. Epub 10/09/2017.

The complex genetics of hypoplastic left heart syndrome.

Liu X, Yagi H, Saeed S, Bais AS, Gabriel GC, Chen Z, Peterson KA, Li Y, Schwartz MC, Reynolds WT, Saydmohammed M, Gibbs B, Wu Y, Devine W, Chatterjee B, Klena NT, Kostka D, de Mesy Bentley KL, Ganapathiraju MK, Dexheimer P, Leatherbury L, Khalifa O, Bhagat A, Zahid M, Pu W, Watkins S, Grossfeld P, Murray SA, Porter GA, Tsang M, Martin LJ, Woodrow Benson D, Aronow BJ, Lo CW

Nature genetics.. 2017 July 49 (7):1152-1159. Epub 05/22/2017.

Analyzing Supercomplexes of the Mitochondrial Electron Transport Chain with Native Electrophoresis, In-gel Assays, and Electroelution.

Beutner G, Porter GA

Journal of visualized experiments : JoVE.. 2017 June 1 (124)Epub 06/01/2017.

Preventing permeability transition pore opening increases mitochondrial maturation, myocyte differentiation and cardiac function in the neonatal mouse heart.

Lingan JV, Alanzalon RE, Porter GA

Pediatric research.. 2017 June 81 (6):932-941. Epub 01/31/2017.

Physiological roles of the mitochondrial permeability transition pore.

Mnatsakanyan N, Beutner G, Porter GA, Alavian KN, Jonas EA

Journal of bioenergetics and biomembranes.. 2017 February 49 (1):13-25. Epub 02/11/2016.

Neonatal hyperoxia depletes pulmonary vein cardiomyocytes in adult mice via mitochondrial oxidation

Yee, M.; Cohen, ED.; Domm, W.; Porter Jr, GA.; McDavid, A.; O'Reilly, MA.

American Journal of Physiology-Lung Cellular and Molecular Physiology. 2017; .

The Mitochondrial Permeability Transition Pore and ATP Synthase.

Beutner G, Kambiz AN, Jonas EA, Porter GA

Handbook of experimental pharmacology.. 2017 240 :21-46. Epub 1900 01 01.

Extraembryonic but not embryonic SUMO-specific protease 2 is required for heart development.

Maruyama EO, Lin H, Chiu SY, Yu HM, Porter GA, Hsu W

Scientific reports.. 2016 February 176 :20999. Epub 02/17/2016.

De novo mutations in congenital heart disease with neurodevelopmental and other congenital anomalies.

Homsy J, Zaidi S, Shen Y, Ware JS, Samocha KE, Karczewski KJ, DePalma SR, McKean D, Wakimoto H, Gorham J, Jin SC, Deanfield J, Giardini A, Porter GA, Kim R, Bilguvar K, López-Giráldez F, Tikhonova I, Mane S, Romano-Adesman A, Qi H, Vardarajan B, Ma L, Daly M, Roberts AE, Russell MW, Mital S, Newburger JW, Gaynor JW, Breitbart RE, Iossifov I, Ronemus M, Sanders SJ, Kaltman JR, Seidman JG, Brueckner M, Gelb BD, Goldmuntz E, Lifton RP, Seidman CE, Chung WK

Science.. 2015 December 4350 (6265):1262-6. Epub 1900 01 01.

Cell death disguised: The mitochondrial permeability transition pore as the c-subunit of the F(1)F(O) ATP synthase.

Jonas EA, Porter GA, Beutner G, Mnatsakanyan N, Alavian KN

Pharmacological research.. 2015 September 99 :382-92. Epub 05/05/2015.

An uncoupling channel within the c-subunit ring of the F1FO ATP synthase is the mitochondrial permeability transition pore.

Alavian KN, Beutner G, Lazrove E, Sacchetti S, Park HA, Licznerski P, Li H, Nabili P, Hockensmith K, Graham M, Porter GA, Jonas EA

Proceedings of the National Academy of Sciences of the United States of America.. 2014 July 22111 (29):10580-5. Epub 06/16/2014.

SIRT3 deficiency exacerbates ischemia-reperfusion injury: implication for aged hearts.

Porter GA, Urciuoli WR, Brookes PS, Nadtochiy SM

American journal of physiology. Heart and circulatory physiology.. 2014 June 15306 (12):H1602-9. Epub 04/18/2014.

Bcl-xL in neuroprotection and plasticity.

Jonas EA, Porter GA, Alavian KN

Frontiers in physiology.. 2014 5 :355. Epub 09/17/2014.

Initiation of electron transport chain activity in the embryonic heart coincides with the activation of mitochondrial complex 1 and the formation of supercomplexes.

Beutner G, Eliseev RA, Porter GA

PloS one.. 2014 9 (11):e113330. Epub 11/26/2014.

De novo mutations in histone-modifying genes in congenital heart disease.

Zaidi S, Choi M, Wakimoto H, Ma L, Jiang J, Overton JD, Romano-Adesman A, Bjornson RD, Breitbart RE, Brown KK, Carriero NJ, Cheung YH, Deanfield J, DePalma S, Fakhro KA, Glessner J, Hakonarson H, Italia MJ, Kaltman JR, Kaski J, Kim R, Kline JK, Lee T, Leipzig J, Lopez A, Mane SM, Mitchell LE, Newburger JW, Parfenov M, Pe'er I, Porter G, Roberts AE, Sachidanandam R, Sanders SJ, Seiden HS, State MW, Subramanian S, Tikhonova IR, Wang W, Warburton D, White PS, Williams IA, Zhao H, Seidman JG, Brueckner M, Chung WK, Gelb BD, Goldmuntz E, Seidman CE, Lifton RP

Nature.. 2013 June 13498 (7453):220-3. Epub 05/12/2013.

The Congenital Heart Disease Genetic Network Study: rationale, design, and early results.

, Gelb B, Brueckner M, Chung W, Goldmuntz E, Kaltman J, Kaski JP, Kim R, Kline J, Mercer-Rosa L, Porter G, Roberts A, Rosenberg E, Seiden H, Seidman C, Sleeper L, Tennstedt S, Kaltman J, Schramm C, Burns K, Pearson G, Rosenberg E

Circulation research.. 2013 February 15112 (4):698-706. Epub 1900 01 01.

Mitochondria as a drug target in ischemic heart disease and cardiomyopathy.

Walters AM, Porter GA, Brookes PS

Circulation research.. 2012 October 12111 (9):1222-36. Epub 1900 01 01.

The permeability transition pore controls cardiac mitochondrial maturation and myocyte differentiation.

Hom JR, Quintanilla RA, Hoffman DL, de Mesy Bentley KL, Molkentin JD, Sheu SS, Porter GA

Developmental cell.. 2011 September 1321 (3):469-78. Epub 1900 01 01.

Bioenergetics, mitochondria, and cardiac myocyte differentiation.

Porter GA, Hom J, Hoffman D, Quintanilla R, de Mesy Bentley K, Sheu SS

Progress in pediatric cardiology.. 2011 May 31 (2):75-81. Epub 1900 01 01.

Regulation of mitochondrial fission by intracellular Ca2+ in rat ventricular myocytes.

Hom J, Yu T, Yoon Y, Porter G, Sheu SS

Biochimica et biophysica acta.. 2010 1797 (6-7):913-21. Epub 03/27/2010.

Caspases 3 and 7: key mediators of mitochondrial events of apoptosis.

Lakhani SA, Masud A, Kuida K, Porter GA, Booth CJ, Mehal WZ, Inayat I, Flavell RA

Science.. 2006 February 10311 (5762):847-51. Epub 1900 01 01.

Intracellular calcium plays an essential role in cardiac development.

Porter GA, Makuck RF, Rivkees SA

Developmental dynamics : an official publication of the American Association of Anatomists.. 2003 June 227 (2):280-90. Epub 1900 01 01.

Reduction in intracellular calcium levels inhibits myoblast differentiation.

Porter GA, Makuck RF, Rivkees SA

The Journal of biological chemistry.. 2002 August 9277 (32):28942-7. Epub 05/31/2002.

Neuregulin-1 promotes formation of the murine cardiac conduction system.

Rentschler S, Zander J, Meyers K, France D, Levine R, Porter G, Rivkees SA, Morley GE, Fishman GI

Proceedings of the National Academy of Sciences of the United States of America.. 2002 August 699 (16):10464-9. Epub 07/29/2002.

Ontogeny of humoral heart rate regulation in the embryonic mouse.

Porter GA, Rivkees SA

American journal of physiology. Regulatory, integrative and comparative physiology.. 2001 August 281 (2):R401-7. Epub 1900 01 01.

Two populations of beta-spectrin in rat skeletal muscle.

Porter GA, Scher MG, Resneck WG, Porter NC, Fowler VM, Bloch RJ

Cell motility and the cytoskeleton.. 1997 37 (1):7-19. Epub 1900 01 01.

Dystrophin colocalizes with beta-spectrin in distinct subsarcolemmal domains in mammalian skeletal muscle.

Porter GA, Dmytrenko GM, Winkelmann JC, Bloch RJ

The Journal of cell biology.. 1992 June 117 (5):997-1005. Epub 1900 01 01.

Heat modifiability of the Escherichia coli iron-regulated membrane proteins.

Murphy, CK.; Porter, GA.; Klebba, PE.

Membrane Proteins, Proceedings of the Membrane Protein Symposium. 1987; : 149-160.

Neonatal hyperoxia depletes pulmonary vein cardiomyocytes in adult mice via mitochondrial oxidation. American Journal of Physiology-Lung Cellular and Molecular Physiology. In revision.

Yee, M.; Cohen, ED.; Domm, W.; Porter, Jr GA.; McDavid, A.; O'Reilly, MA.

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