Year |
Citation |
Score |
2023 |
Schweitzer GG, Ditzenberger GL, Hughey CC, Finck BN, Martino MR, Pacak CA, Byrne BJ, Cade WT. Elevated liver glycogenolysis mediates higher blood glucose during acute exercise in Barth syndrome. Plos One. 18: e0290832. PMID 37651450 DOI: 10.1371/journal.pone.0290832 |
0.482 |
|
2021 |
Bohnert KL, Ditzenberger G, Bittel AJ, de Las Fuentes L, Corti M, Pacak CA, Taylor C, Byrne BJ, Reeds DN, Cade WT. Resistance exercise training with protein supplementation improves skeletal muscle strength and improves quality of life in late adolescents and young adults with Barth syndrome: A pilot study. Jimd Reports. 62: 74-84. PMID 34765401 DOI: 10.1002/jmd2.12244 |
0.473 |
|
2020 |
Cade WT, Bohnert KL, Bittel AJ, Chacko SJ, Patterson BW, Pacak CA, Byrne BJ, Vernon HJ, Reeds DN. Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome. Molecular Genetics and Metabolism Reports. 25: 100675. PMID 33204638 DOI: 10.1016/j.ymgmr.2020.100675 |
0.422 |
|
2020 |
Barton ER, Pacak CA, Stoppel WL, Kang PB. The ties that bind: functional clusters in limb-girdle muscular dystrophy. Skeletal Muscle. 10: 22. PMID 32727611 DOI: 10.1186/S13395-020-00240-7 |
0.433 |
|
2020 |
Watanabe N, Kitada K, Santostefano KE, Yokoyama A, Waldrop SM, Heldermon CD, Tachibana D, Koyama M, Meacham AM, Pacak CA, Terada N. Generation of Induced Pluripotent Stem Cells from a Female Patient with a Xq27.3-q28 Deletion to Establish Disease Models and Identify Therapies. Cellular Reprogramming. PMID 32608992 DOI: 10.1089/Cell.2020.0012 |
0.317 |
|
2020 |
Suzuki-Hatano S, Tsai AC, Daugherty A, Pacak CA. TMT Sample Preparation for Proteomics Facility Submission and Subsequent Data Analysis. Journal of Visualized Experiments : Jove. PMID 32568242 DOI: 10.3791/60970 |
0.305 |
|
2020 |
Bolfer L, Estrada AH, Larkin C, Conlon TJ, Lourenco F, Taggart K, Suzuki-Hatano S, Pacak CA. Functional Consequences of PDK4 Deficiency in Doberman Pinscher Fibroblasts. Scientific Reports. 10: 3930. PMID 32127618 DOI: 10.1038/S41598-020-60879-6 |
0.361 |
|
2019 |
Cade WT, Laforest R, Bohnert KL, Reeds DN, Bittel AJ, de Las Fuentes L, Bashir A, Woodard PK, Pacak CA, Byrne BJ, Gropler RJ, Peterson LR. Myocardial glucose and fatty acid metabolism is altered and associated with lower cardiac function in young adults with Barth syndrome. Journal of Nuclear Cardiology : Official Publication of the American Society of Nuclear Cardiology. PMID 31705425 DOI: 10.1007/S12350-019-01933-3 |
0.537 |
|
2019 |
Suzuki-Hatano S, Sriramvenugopal M, Ramanathan M, Soustek M, Byrne BJ, Cade WT, Kang PB, Pacak CA. Increased mtDNA Abundance and Improved Function in Human Barth Syndrome Patient Fibroblasts Following AAV- Gene Delivery. International Journal of Molecular Sciences. 20. PMID 31336787 DOI: 10.3390/Ijms20143416 |
0.562 |
|
2019 |
Saha M, Rizzo SA, Ramanathan M, Hightower RM, Santostefano KE, Terada N, Finkel RS, Berg JS, Chahin N, Pacak CA, Wagner RE, Alexander MS, Draper I, Kang PB. Selective serotonin reuptake inhibitors ameliorate MEGF10 myopathy. Human Molecular Genetics. 28: 2365-2377. PMID 31267131 DOI: 10.1093/Hmg/Ddz064 |
0.403 |
|
2019 |
Cade WT, Bohnert KL, Peterson LR, Patterson BW, Bittel AJ, Okunade AL, de Las Fuentes L, Steger-May K, Bashir A, Schweitzer GG, Chacko SK, Wanders RJ, Pacak CA, Byrne BJ, Reeds DN. Blunted Fat Oxidation upon Submaximal Exercise is Partially Compensated by Enhanced Glucose Metabolism in Children, Adolescents and Young Adults with Barth Syndrome. Journal of Inherited Metabolic Disease. PMID 30924938 DOI: 10.1002/Jimd.12094 |
0.492 |
|
2019 |
Suzuki-Hatano S, Saha M, Soustek MS, Kang PB, Byrne BJ, Cade WT, Pacak CA. AAV9- Gene Replacement Ameliorates Cardiac TMT Proteomic Profiles in a Mouse Model of Barth Syndrome. Molecular Therapy. Methods & Clinical Development. 13: 167-179. PMID 30788385 DOI: 10.1016/J.Omtm.2019.01.007 |
0.615 |
|
2018 |
Saha M, Reddy HM, Salih MA, Estrella E, Jones MD, Mitsuhashi S, Cho KA, Suzuki-Hatano S, Rizzo SA, Hamad MH, Mukhtar MM, Hamed AA, Elseed MA, Lek M, Valkanas E, ... ... Pacak CA, et al. Impact of PYROXD1 deficiency on cellular respiration and correlations with genetic analyses of limb-girdle muscular dystrophy in Saudi Arabia and Sudan. Physiological Genomics. 50: 929-939. PMID 30345904 DOI: 10.1152/Physiolgenomics.00036.2018 |
0.325 |
|
2018 |
Suzuki-Hatano S, Saha M, Rizzo SA, Witko RL, Gosiker BJ, Ramanathan M, Soustek MS, Jones MD, Kang PB, Byrne BJ, Cade WT, Pacak CA. AAV-Mediated TAZ Gene Replacement Restores Mitochondrial and Cardioskeletal Function in Barth Syndrome. Human Gene Therapy. PMID 30070157 DOI: 10.1089/Hum.2018.020 |
0.647 |
|
2017 |
Taggart K, Estrada A, Thompson P, Lourenco F, Kirmani S, Suzuki-Hatano S, Pacak CA. PDK4 Deficiency Induces Intrinsic Apoptosis in Response to Starvation in Fibroblasts from Doberman Pinschers with Dilated Cardiomyopathy. Bioresearch Open Access. 6: 182-191. PMID 29285418 DOI: 10.1089/Biores.2017.0023 |
0.363 |
|
2017 |
Saha M, Mitsuhashi S, Jones MD, Manko K, Reddy HM, Bruels C, Cho KA, Pacak CA, Draper I, Kang PB. Consequences of MEGF10 deficiency on myoblast function and Notch1 interactions. Human Molecular Genetics. PMID 28498977 DOI: 10.1093/Hmg/Ddx189 |
0.365 |
|
2017 |
Bashir A, Bohnert KL, Reeds DN, Peterson LR, Bittel AJ, de Las Fuentes L, Pacak CA, Byrne BJ, Cade WT. Impaired cardiac and skeletal muscle bioenergetics in children, adolescents, and young adults with Barth syndrome. Physiological Reports. 5. PMID 28196853 DOI: 10.14814/Phy2.13130 |
0.553 |
|
2016 |
Conlon TJ, Mah CS, Pacak CA, Rucker Henninger MB, Erger KE, Jorgensen ML, Lee CC, Tarantal AF, Byrne BJ. Transfer of Therapeutic Genes into Fetal Rhesus Monkeys using Recombinant Adeno-Associated Type I Viral Vectors. Human Gene Therapy. Clinical Development. PMID 27855487 DOI: 10.1089/Humc.2016.119 |
0.632 |
|
2016 |
Rizzo S, Donate AL, Jones M, Manko K, Santostefano KE, Terada N, Alkuraya FS, Kang PB, Pacak CA. 360. Genotype/Phenotype Correlation of Cellular Function and AAV-Mediated Gene Delivery to Treat Xeroderma Pigmentosum - Cockayne Syndrome (XP - CS) Molecular Therapy. 24: S144. DOI: 10.1016/S1525-0016(16)33169-0 |
0.397 |
|
2016 |
Gosiker B, Rizzo S, Donate A, Pacak C. 358. AAV-MitoTimer to Asses Mitochondrial Turnover Molecular Therapy. 24: S143. DOI: 10.1016/S1525-0016(16)33167-7 |
0.366 |
|
2016 |
Bolfer L, Estrada AH, Winter B, Taggart K, Lourenço F, Terada N, Byrne BJ, Conlon TJ, Pacak CA. 180. The Role of the Pyruvate Dehydrogenase Kinase 4 (PDK4) Gene Mutation in the Development of Dilated Cardiomyopathy in Dobermanpinschers and Potential Application of Cardiac Gene Therapy Molecular Therapy. 24: S70-S71. DOI: 10.1016/S1525-0016(16)32989-6 |
0.6 |
|
2015 |
Pacak CA, Preble JM, Kondo H, Seibel P, Levitsky S, Del Nido PJ, Cowan DB, McCully JD. Actin-dependent mitochondrial internalization in cardiomyocytes: evidence for rescue of mitochondrial function. Biology Open. 4: 622-6. PMID 25862247 DOI: 10.1242/Bio.201511478 |
0.313 |
|
2015 |
Love-Leonor T, Corti M, Boye S, Donate A, Santostefano K, Cloutier D, Clemente N, Pacak C, Byrne B. 191. Frataxin: A Putative Biomarker for Minimal Effective Dosage of AAV Gene Therapy Molecular Therapy. 23: S76. DOI: 10.1016/S1525-0016(16)33796-0 |
0.607 |
|
2015 |
Donate AL, Soustek M, Rizzo SA, Santostefano K, Gosiker BJ, Lewin AS, Terada N, Cade T, Byrne BJ, Pacak CA. 187. AAV Delivery of Tafazzin for the Correction of Mitochondrial Dysfunction in Barth Syndrome Using IPS Differentiated Cardiomyocytes Molecular Therapy. 23: S75. DOI: 10.1016/S1525-0016(16)33792-3 |
0.608 |
|
2015 |
Rizzo SA, Donate AL, Gosiker BJ, Santostefano KE, Terada N, Alkuraya FS, Kang PB, Pacak CA. 182. Characterization and AAV-Mediated Correction of Xeroderma Pigmentosum-Cockayne Syndrome (XP/CS) Molecular Therapy. 23: S73. DOI: 10.1016/S1525-0016(16)33787-X |
0.378 |
|
2014 |
Draper I, Mahoney LJ, Mitsuhashi S, Pacak CA, Salomon RN, Kang PB. Silencing of drpr leads to muscle and brain degeneration in adult Drosophila. The American Journal of Pathology. 184: 2653-61. PMID 25111228 DOI: 10.1016/J.Ajpath.2014.06.018 |
0.399 |
|
2013 |
Masuzawa A, Black KM, Pacak CA, Ericsson M, Barnett RJ, Drumm C, Seth P, Bloch DB, Levitsky S, Cowan DB, McCully JD. Transplantation of autologously derived mitochondria protects the heart from ischemia-reperfusion injury. American Journal of Physiology. Heart and Circulatory Physiology. 304: H966-82. PMID 23355340 DOI: 10.1152/Ajpheart.00883.2012 |
0.353 |
|
2011 |
Pacak CA, Byrne BJ. AAV vectors for cardiac gene transfer: experimental tools and clinical opportunities. Molecular Therapy : the Journal of the American Society of Gene Therapy. 19: 1582-90. PMID 21792180 DOI: 10.1038/Mt.2011.124 |
0.626 |
|
2011 |
Byrne BJ, Falk DJ, Pacak CA, Nayak S, Herzog RW, Elder ME, Collins SW, Conlon TJ, Clement N, Cleaver BD, Cloutier DA, Porvasnik SL, Islam S, Elmallah MK, Martin A, et al. Pompe disease gene therapy. Human Molecular Genetics. 20: R61-8. PMID 21518733 DOI: 10.1093/Hmg/Ddr174 |
0.579 |
|
2010 |
Mah CS, Pacak CA, Byrne BJ. Muscle as a metabolic factory for gene therapy Muscle Gene Therapy. 219-230. DOI: 10.1007/978-1-4419-1207-7_13 |
0.311 |
|
2009 |
Sill B, Alpatov IV, Pacak CA, Cowan DB. Implantation of engineered tissue in the rat heart. Journal of Visualized Experiments : Jove. PMID 19553905 DOI: 10.3791/1139 |
0.306 |
|
2009 |
Pacak CA, Cowan DB. Fabrication of myogenic engineered tissue constructs. Journal of Visualized Experiments : Jove. PMID 19412158 DOI: 10.3791/1137 |
0.338 |
|
2009 |
McCully JD, Cowan DB, Pacak CA, Toumpoulis IK, Dayalan H, Levitsky S. Injection of isolated mitochondria during early reperfusion for cardioprotection. American Journal of Physiology. Heart and Circulatory Physiology. 296: H94-H105. PMID 18978192 DOI: 10.1152/Ajpheart.00567.2008 |
0.307 |
|
2008 |
Pacak CA, Conlon T, Mah CS, Byrne BJ. Relative persistence of AAV serotype 1 vector genomes in dystrophic muscle. Genetic Vaccines and Therapy. 6: 14. PMID 18854054 DOI: 10.1186/1479-0556-6-14 |
0.609 |
|
2008 |
Pacak CA, Sakai Y, Thattaliyath BD, Mah CS, Byrne BJ. Tissue specific promoters improve specificity of AAV9 mediated transgene expression following intra-vascular gene delivery in neonatal mice. Genetic Vaccines and Therapy. 6: 13. PMID 18811960 DOI: 10.1186/1479-0556-6-13 |
0.632 |
|
2008 |
Foust KD, Poirier A, Pacak CA, Mandel RJ, Flotte TR. Neonatal intraperitoneal or intravenous injections of recombinant adeno-associated virus type 8 transduce dorsal root ganglia and lower motor neurons. Human Gene Therapy. 19: 61-70. PMID 18052722 DOI: 10.1089/Hum.2007.093 |
0.343 |
|
2007 |
Pacak CA, Walter GA, Gaidosh G, Bryant N, Lewis MA, Germain S, Mah CS, Campbell KP, Byrne BJ. Long-term skeletal muscle protection after gene transfer in a mouse model of LGMD-2D. Molecular Therapy : the Journal of the American Society of Gene Therapy. 15: 1775-81. PMID 17653106 DOI: 10.1038/Sj.Mt.6300246 |
0.604 |
|
2007 |
Mah C, Pacak CA, Cresawn KO, Deruisseau LR, Germain S, Lewis MA, Cloutier DA, Fuller DD, Byrne BJ. Physiological correction of Pompe disease by systemic delivery of adeno-associated virus serotype 1 vectors. Molecular Therapy : the Journal of the American Society of Gene Therapy. 15: 501-7. PMID 17245350 DOI: 10.1038/Sj.Mt.6300100 |
0.767 |
|
2006 |
Pacak CA, Mah CS, Thattaliyath BD, Conlon TJ, Lewis MA, Cloutier DE, Zolotukhin I, Tarantal AF, Byrne BJ. Recombinant adeno-associated virus serotype 9 leads to preferential cardiac transduction in vivo. Circulation Research. 99: e3-9. PMID 16873720 DOI: 10.1161/01.Res.0000237661.18885.F6 |
0.653 |
|
2006 |
Mah C, DeRuisseau LR, Pacak CA, Lewis MA, Fuller DD, Byrne BJ. 575. Correction of Ventilation in Glycogen Storage Disease Type II Mice after Gel-Mediated Delivery of Adeno-Associated Virus Serotype 1 Vectors Molecular Therapy. 13: S221-S222. DOI: 10.1016/J.Ymthe.2006.08.648 |
0.77 |
|
2006 |
Mah C, Pacak CA, Cresawn KO, DeRuisseau LR, Germain S, Lewis MA, Fuller DD, Byrne BJ. 410. Physiological Correction of Glycogen Storage Disease Type II Using Adeno-Associated Virus Serotype 1 Vectors Molecular Therapy. 13. DOI: 10.1016/J.Ymthe.2006.08.473 |
0.768 |
|
2006 |
Pacak CA, Mah C, Cresawn KO, Lewis MA, Germain S, Byrne BJ. 28. rAAV2/9 Mediated Gene Delivery of Acid α-Glucosidase Corrects the Cardiac Phenotype in a Mouse Model of Pompe Disease Molecular Therapy. 13: S12. DOI: 10.1016/J.Ymthe.2006.08.040 |
0.808 |
|
2005 |
Mah C, Cresawn KO, Fraites TJ, Pacak CA, Lewis MA, Zolotukhin I, Byrne BJ. Sustained correction of glycogen storage disease type II using adeno-associated virus serotype 1 vectors Gene Therapy. 12: 1405-1409. PMID 15920463 DOI: 10.1038/Sj.Gt.3302550 |
0.741 |
|
2005 |
Pacak CA, Mah C, Gaidosh G, Lewis M, Torres R, Campbell K, Walter GA, Byrne BJ. 44. Development of AAV-Mediated Gene Therapy for Murine Models of Genetic Diseases Affecting the Heart Molecular Therapy. 11. DOI: 10.1016/J.Ymthe.2005.06.072 |
0.684 |
|
2004 |
Cresawn KO, Raben N, Plotz PH, Pacak C, Glick A, Byrne BJ. 859. A Skin-Restricted Conditionally Expressing Mouse Model of Pompe Disease for Assessment of Gene and Enzyme Replacement Therapies Molecular Therapy. 9. DOI: 10.1016/J.Ymthe.2004.06.767 |
0.767 |
|
2004 |
Pacak CA, Cloutier D, Zolotukhin I, Gaidosh GS, Campbell K, Walter GA, Byrne BJ. 251. rAAV-Mediated Gene Therapy To Treat Limb Girdle Muscular Dystrophy Type 2D (LGMD-2D) Molecular Therapy. 9. DOI: 10.1016/J.Ymthe.2004.06.192 |
0.605 |
|
2003 |
Schnepp BC, Clark KR, Klemanski DL, Pacak CA, Johnson PR. Genetic fate of recombinant adeno-associated virus vector genomes in muscle. Journal of Virology. 77: 3495-504. PMID 12610125 DOI: 10.1128/Jvi.77.6.3495-3504.2003 |
0.389 |
|
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