Search Results - "Porter, George A."

uncoupling channel within the c-subunit ring of the F₁FO ATP synthase is the mitochondrial permeability transition pore by Alavian, Kambiz N, Beutner, Gisela, Lazrove, Emma, Sacchetti, Silvio, Park, Han-A, Licznerski, Pawel, Li, Hongmei, Nabili, Panah, Hockensmith, Kathryn, Graham, Morven, Porter, Jr, George A, Jonas, Elizabeth A

“…Mitochondria maintain tight regulation of inner mitochondrial membrane (IMM) permeability to sustain ATP production. Stressful events cause cellular calcium…”
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Mitochondria as a Drug Target in Ischemic Heart Disease and Cardiomyopathy by Walters, Andrew M, Porter, George A, Brookes, Paul S

“…Ischemic heart disease is a significant cause of morbidity and mortality in Western society. Although interventions, such as thrombolysis and percutaneous…”
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Cyclophilin D, Somehow a Master Regulator of Mitochondrial Function by Porter, Jr, George A, Beutner, Gisela

“…Cyclophilin D (CyPD) is an important mitochondrial chaperone protein whose mechanism of action remains a mystery. It is well known for regulating mitochondrial…”
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Environmental Signals by Porter, Jr, George A

“…Environmental factors have long been known to play a role in the pathogenesis of congenital heart disease (CHD), but this has not been a major focus of…”
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The Permeability Transition Pore Controls Cardiac Mitochondrial Maturation and Myocyte Differentiation by Hom, Jennifer R., Quintanilla, Rodrigo A., Hoffman, David L., de Mesy Bentley, Karen L., Molkentin, Jeffery D., Sheu, Shey-Shing, Porter, George A.

“…Although mature myocytes rely on mitochondria as the primary source of energy, the role of mitochondria in the developing heart is not well known. Here, we…”
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SIRT3 deficiency exacerbates ischemia-reperfusion injury: implication for aged hearts by Porter, George A, Urciuoli, William R, Brookes, Paul S, Nadtochiy, Sergiy M

“…Ischemia-reperfusion (IR) injury is significantly worse in aged hearts, but the underlying mechanisms are poorly understood. Age-related damage to mitochondria…”
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The complex genetics of hypoplastic left heart syndrome by Liu, Xiaoqin, Yagi, Hisato, Saeed, Shazina, Bais, Abha S, Gabriel, George C, Chen, Zhaohan, Peterson, Kevin A, Li, You, Schwartz, Molly C, Reynolds, William T, Saydmohammed, Manush, Gibbs, Brian, Wu, Yijen, Devine, William, Chatterjee, Bishwanath, Klena, Nikolai T, Kostka, Dennis, de Mesy Bentley, Karen L, Ganapathiraju, Madhavi K, Dexheimer, Phillip, Leatherbury, Linda, Khalifa, Omar, Bhagat, Anchit, Zahid, Maliha, Pu, William, Watkins, Simon, Grossfeld, Paul, Murray, Stephen A, Porter, George A, Tsang, Michael, Martin, Lisa J, Benson, D Woodrow, Aronow, Bruce J, Lo, Cecilia W

“…Cecilia Lo and colleagues report the recovery of mice with hypoplastic left heart syndrome (HLHS) from a large mutagenesis screen. They find genetic…”
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Caspases 3 and 7: Key Mediators of Mitochondrial Events of Apoptosis by Lakhani, Saquib A., Masud, Ali, Kuida, Keisuke, Porter, George A., Booth, Carmen J., Menal, Wajahat Z., Inayat, Irteza, Flavell, Richard A.

“…The current model of apoptosis holds that upstream signals lead to activation of downstream effector caspases. We generated mice deficient in the two…”
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Physiological roles of the mitochondrial permeability transition pore by Mnatsakanyan, Nelli, Beutner, Gisela, Porter, George A., Alavian, Kambiz N., Jonas, Elizabeth A.

“…Neurons experience high metabolic demand during such processes as synaptic vesicle recycling, membrane potential maintenance and Ca 2+ exchange/extrusion. The…”
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The Congenital Heart Disease Genetic Network Study: Cohort description by Hoang, Thanh T, Goldmuntz, Elizabeth, Roberts, Amy E, Chung, Wendy K, Kline, Jennie K, Deanfield, John E, Giardini, Alessandro, Aleman, Adolfo, Gelb, Bruce D, Mac Neal, Meghan, Porter, Jr, George A, Kim, Richard, Brueckner, Martina, Lifton, Richard P, Edman, Sharon, Woyciechowski, Stacy, Mitchell, Laura E, Agopian, A J

“…The Pediatric Cardiac Genomics Consortium (PCGC) designed the Congenital Heart Disease Genetic Network Study to provide phenotype and genotype data for a large…”
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Cyclophilin D regulates the dynamic assembly of mitochondrial ATP synthase into synthasomes by Beutner, Gisela, Alanzalon, Ryan E., Porter, George A.

“…Mitochondrial electron transport is essential for oxidative phosphorylation (OXPHOS). Electron transport chain (ETC) activity generates an electrochemical…”
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Dual role of inorganic polyphosphate in cardiac myocytes: The importance of polyP chain length for energy metabolism and mPTP activation by Seidlmayer, Lea K., Gomez-Garcia, Maria R., Shiba, Toshikazu, Porter, George A., Pavlov, Evgeny V., Bers, Donald M., Dedkova, Elena N.

“…We have previously demonstrated that inorganic polyphosphate (polyP) is a potent activator of the mitochondrial permeability transition pore (mPTP) in cardiac…”
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Role of the Mitochondrial Permeability Transition in Bone Metabolism and Aging by Sautchuk, Rubens, Yu, Chen, McArthur, Matthew, Massie, Christine, Brookes, Paul S., Porter, George A., Awad, Hani, Eliseev, Roman A.

“…ABSTRACT The mitochondrial permeability transition pore (MPTP) and its positive regulator, cyclophilin D (CypD), play important pathophysiological roles in…”
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A reversible mitochondrial complex I thiol switch mediates hypoxic avoidance behavior in C. elegans by Onukwufor, John O., Farooqi, M. Arsalan, Vodičková, Anežka, Koren, Shon A., Baldzizhar, Aksana, Berry, Brandon J., Beutner, Gisela, Porter, George A., Belousov, Vsevolod, Grossfield, Alan, Wojtovich, Andrew P.

“…C. elegans react to metabolic distress caused by mismatches in oxygen and energy status via distinct behavioral responses. At the molecular level, these…”
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Caspase-3 cleaved tau impairs mitochondrial function through the opening of the mitochondrial permeability transition pore by Pérez, María José, Ibarra-García-Padilla, Rodrigo, Tang, Maoping, Porter, George A., Johnson, Gail V.W., Quintanilla, Rodrigo A.

“…Mitochondrial dysfunction is a significant factor in the development of Alzheimer's disease (AD). Previous studies have demonstrated that the expression of tau…”
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PPARγ drives mitochondrial stress signaling and the loss of atrial cardiomyocytes in newborn mice exposed to hyperoxia by Cohen, E. David, Roethlin, Kyle, Yee, Min, Woeller, Collynn F., Brookes, Paul S., Porter, George A., O'Reilly, Michael A.

“…Diastolic dysfunction is increasingly common in preterm infants exposed to supplemental oxygen (hyperoxia). Previous studies in neonatal mice showed hyperoxia…”
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Initiation of electron transport chain activity in the embryonic heart coincides with the activation of mitochondrial complex 1 and the formation of supercomplexes by Beutner, Gisela, Eliseev, Roman A, Porter, Jr, George A

“…Mitochondria provide energy in form of ATP in eukaryotic cells. However, it is not known when, during embryonic cardiac development, mitochondria become able…”
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Neonatal hyperoxia depletes pulmonary vein cardiomyocytes in adult mice via mitochondrial oxidation by Yee, Min, Cohen, Ethan David, Domm, William, Porter, Jr, George A, McDavid, Andrew N, O'Reilly, Michael A

“…Supplemental oxygen given to preterm infants has been associated with permanently altering postnatal lung development. Now that these individuals are reaching…”
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Genomic analyses implicate noncoding de novo variants in congenital heart disease by Richter, Felix, Morton, Sarah U., Kim, Seong Won, Kitaygorodsky, Alexander, Wasson, Lauren K., Chen, Kathleen M., Zhou, Jian, Qi, Hongjian, Patel, Nihir, DePalma, Steven R., Parfenov, Michael, Homsy, Jason, Gorham, Joshua M., Manheimer, Kathryn B., Velinder, Matthew, Farrell, Andrew, Marth, Gabor, Schadt, Eric E., Kaltman, Jonathan R., Newburger, Jane W., Giardini, Alessandro, Goldmuntz, Elizabeth, Brueckner, Martina, Kim, Richard, Porter, George A., Bernstein, Daniel, Chung, Wendy K., Srivastava, Deepak, Tristani-Firouzi, Martin, Troyanskaya, Olga G., Dickel, Diane E., Shen, Yufeng, Seidman, Jonathan G., Seidman, Christine E., Gelb, Bruce D.

“…A genetic etiology is identified for one-third of patients with congenital heart disease (CHD), with 8% of cases attributable to coding de novo variants…”
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Potassium conservation is impaired in mice with reduced renal expression of Kir4.1 by Malik, Sundeep, Lambert, Emily, Zhang, Junhui, Wang, Tong, Clark, Heather L, Cypress, Michael, Goldman, Bruce I, Porter, Jr, George A, Pena, Salvador, Nino, Wilson, Gray, Daniel A

“…To better understand the role of the inward-rectifying K channel Kir4.1 (KCNJ10) in the distal nephron, we initially studied a global Kir4.1 knockout mouse…”
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