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Items: 37

1.

Blunted fat oxidation upon submaximal exercise is partially compensated by enhanced glucose metabolism in children, adolescents, and young adults with Barth syndrome.

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.

J Inherit Metab Dis. 2019 May;42(3):480-493. doi: 10.1002/jimd.12094. Epub 2019 Apr 11.

PMID:
30924938
2.

AAV9-TAZ Gene Replacement Ameliorates Cardiac TMT Proteomic Profiles in a Mouse Model of Barth Syndrome.

Suzuki-Hatano S, Saha M, Soustek MS, Kang PB, Byrne BJ, Cade WT, Pacak CA.

Mol Ther Methods Clin Dev. 2019 Jan 25;13:167-179. doi: 10.1016/j.omtm.2019.01.007. eCollection 2019 Jun 14.

3.

Identification of a pathogenic mutation in ATP2A1 via in silico analysis of exome data for cryptic aberrant splice sites.

Bruels CC, Li C, Mendoza T, Khan J, Reddy HM, Estrella EA, Ghosh PS, Darras BT, Lidov HGW, Pacak CA, Kunkel LM, Modave F, Draper I, Kang PB.

Mol Genet Genomic Med. 2019 Mar;7(3):e552. doi: 10.1002/mgg3.552. Epub 2019 Jan 28.

4.

Impact of PYROXD1 deficiency on cellular respiration and correlations with genetic analyses of limb-girdle muscular dystrophy in Saudi Arabia and Sudan.

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, MacArthur DG, Kunkel LM, Pacak CA, Draper I, Kang PB.

Physiol Genomics. 2018 Nov 1;50(11):929-939. doi: 10.1152/physiolgenomics.00036.2018. Epub 2018 Aug 31.

PMID:
30345904
5.

Infiltrative and drug-resistant slow-cycling cells support metabolic heterogeneity in glioblastoma.

Hoang-Minh LB, Siebzehnrubl FA, Yang C, Suzuki-Hatano S, Dajac K, Loche T, Andrews N, Schmoll Massari M, Patel J, Amin K, Vuong A, Jimenez-Pascual A, Kubilis P, Garrett TJ, Moneypenny C, Pacak CA, Huang J, Sayour EJ, Mitchell DA, Sarkisian MR, Reynolds BA, Deleyrolle LP.

EMBO J. 2018 Dec 3;37(23). pii: e98772. doi: 10.15252/embj.201798772. Epub 2018 Oct 15.

PMID:
30322894
6.

AAV-Mediated TAZ Gene Replacement Restores Mitochondrial and Cardioskeletal Function in Barth Syndrome.

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.

Hum Gene Ther. 2019 Feb;30(2):139-154. doi: 10.1089/hum.2018.020. Epub 2018 Oct 3.

PMID:
30070157
7.

PDK4 Deficiency Induces Intrinsic Apoptosis in Response to Starvation in Fibroblasts from Doberman Pinschers with Dilated Cardiomyopathy.

Taggart K, Estrada A, Thompson P, Lourenco F, Kirmani S, Suzuki-Hatano S, Pacak CA.

Biores Open Access. 2017 Dec 1;6(1):182-191. doi: 10.1089/biores.2017.0023. eCollection 2017.

8.

Consequences of MEGF10 deficiency on myoblast function and Notch1 interactions.

Saha M, Mitsuhashi S, Jones MD, Manko K, Reddy HM, Bruels CC, Cho KA, Pacak CA, Draper I, Kang PB.

Hum Mol Genet. 2017 Aug 1;26(15):2984-3000. doi: 10.1093/hmg/ddx189.

9.

Impaired cardiac and skeletal muscle bioenergetics in children, adolescents, and young adults with Barth syndrome.

Bashir A, Bohnert KL, Reeds DN, Peterson LR, Bittel AJ, de Las Fuentes L, Pacak CA, Byrne BJ, Cade WT.

Physiol Rep. 2017 Feb;5(3). pii: e13130. doi: 10.14814/phy2.13130.

10.

Transfer of Therapeutic Genes into Fetal Rhesus Monkeys Using Recombinant Adeno-Associated Type I Viral Vectors.

Conlon TJ, Mah CS, Pacak CA, Rucker Henninger MB, Erger KE, Jorgensen ML, Lee CC, Tarantal AF, Byrne BJ.

Hum Gene Ther Clin Dev. 2016 Dec;27(4):152-159.

11.

Longitudinal Patterns of Thalidomide Neuropathy in Children and Adolescents.

Liew WK, Pacak CA, Visyak N, Darras BT, Bousvaros A, Kang PB.

J Pediatr. 2016 Nov;178:227-232. doi: 10.1016/j.jpeds.2016.07.040. Epub 2016 Aug 24.

PMID:
27567409
12.

(18)F-labeled rhodamines as potential myocardial perfusion agents: comparison of pharmacokinetic properties of several rhodamines.

Bartholomä MD, Zhang S, Akurathi V, Pacak CA, Dunning P, Fahey FH, Cowan DB, Treves ST, Packard AB.

Nucl Med Biol. 2015 Oct;42(10):796-803. doi: 10.1016/j.nucmedbio.2015.06.008. Epub 2015 Jun 18.

13.

Actin-dependent mitochondrial internalization in cardiomyocytes: evidence for rescue of mitochondrial function.

Pacak CA, Preble JM, Kondo H, Seibel P, Levitsky S, Del Nido PJ, Cowan DB, McCully JD.

Biol Open. 2015 Apr 10;4(5):622-6. doi: 10.1242/bio.201511478.

14.

Superparamagnetic iron oxide nanoparticles function as a long-term, multi-modal imaging label for non-invasive tracking of implanted progenitor cells.

Pacak CA, Hammer PE, MacKay AA, Dowd RP, Wang KR, Masuzawa A, Sill B, McCully JD, Cowan DB.

PLoS One. 2014 Sep 24;9(9):e108695. doi: 10.1371/journal.pone.0108695. eCollection 2014.

15.

Rapid isolation and purification of mitochondria for transplantation by tissue dissociation and differential filtration.

Preble JM, Pacak CA, Kondo H, MacKay AA, Cowan DB, McCully JD.

J Vis Exp. 2014 Sep 6;(91):e51682. doi: 10.3791/51682.

16.

Growth of bone marrow and skeletal muscle side population stem cells in suspension culture.

Pacak CA, Cowan DB.

Methods Mol Biol. 2014;1210:51-61. doi: 10.1007/978-1-4939-1435-7_5.

PMID:
25173160
17.

Silencing of drpr leads to muscle and brain degeneration in adult Drosophila.

Draper I, Mahoney LJ, Mitsuhashi S, Pacak CA, Salomon RN, Kang PB.

Am J Pathol. 2014 Oct;184(10):2653-61. doi: 10.1016/j.ajpath.2014.06.018. Epub 2014 Aug 8.

18.

An improved method for the preparation of type I collagen from skin.

Pacak CA, MacKay AA, Cowan DB.

J Vis Exp. 2014 Jan 21;(83):e51011. doi: 10.3791/51011.

19.

Straightening of curved pattern of collagen fibers under load controls aortic valve shape.

Hammer PE, Pacak CA, Howe RD, del Nido PJ.

J Biomech. 2014 Jan 22;47(2):341-6. doi: 10.1016/j.jbiomech.2013.11.032. Epub 2013 Nov 28.

20.

Biological characterization of F-18-labeled rhodamine B, a potential positron emission tomography perfusion tracer.

Bartholomä MD, He H, Pacak CA, Dunning P, Fahey FH, McGowan FX, Cowan DB, Treves ST, Packard AB.

Nucl Med Biol. 2013 Nov;40(8):1043-8. doi: 10.1016/j.nucmedbio.2013.07.006. Epub 2013 Sep 4.

21.

Microcarrier-based expansion of adult murine side population stem cells.

Pacak CA, Eddy MT, Woodhull L, Wang KR, Alpatov I, Fullen S, Dowd RP, Choi YH, Cowan DB.

PLoS One. 2013;8(1):e55187. doi: 10.1371/journal.pone.0055187. Epub 2013 Jan 28.

22.

Transplantation of autologously derived mitochondria protects the heart from ischemia-reperfusion injury.

Masuzawa A, Black KM, Pacak CA, Ericsson M, Barnett RJ, Drumm C, Seth P, Bloch DB, Levitsky S, Cowan DB, McCully JD.

Am J Physiol Heart Circ Physiol. 2013 Apr 1;304(7):H966-82. doi: 10.1152/ajpheart.00883.2012. Epub 2013 Jan 25.

23.

AAV vectors for cardiac gene transfer: experimental tools and clinical opportunities.

Pacak CA, Byrne BJ.

Mol Ther. 2011 Sep;19(9):1582-90. doi: 10.1038/mt.2011.124. Epub 2011 Jul 26. Review.

24.

Pompe disease gene therapy.

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, Smith BK, Fuller DD, Lawson LA, Mah CS.

Hum Mol Genet. 2011 Apr 15;20(R1):R61-8. doi: 10.1093/hmg/ddr174. Epub 2011 Apr 25. Review.

25.

Ultrarapid purification of collagen type I for tissue engineering applications.

Pacak CA, Powers JM, Cowan DB.

Tissue Eng Part C Methods. 2011 Sep;17(9):879-85. doi: 10.1089/ten.TEC.2010.0720. Epub 2011 Jun 1.

26.

Implantation of engineered tissue in the rat heart.

Sill B, Alpatov IV, Pacak CA, Cowan DB.

J Vis Exp. 2009 Jun 24;(28). pii: 1139. doi: 10.3791/1139.

27.

Fabrication of myogenic engineered tissue constructs.

Pacak CA, Cowan DB.

J Vis Exp. 2009 May 1;(27). pii: 1137. doi: 10.3791/1137.

28.

Injection of isolated mitochondria during early reperfusion for cardioprotection.

McCully JD, Cowan DB, Pacak CA, Toumpoulis IK, Dayalan H, Levitsky S.

Am J Physiol Heart Circ Physiol. 2009 Jan;296(1):H94-H105. doi: 10.1152/ajpheart.00567.2008. Epub 2008 Oct 31.

29.

Relative persistence of AAV serotype 1 vector genomes in dystrophic muscle.

Pacak CA, Conlon T, Mah CS, Byrne BJ.

Genet Vaccines Ther. 2008 Oct 15;6:14. doi: 10.1186/1479-0556-6-14.

30.

Tissue specific promoters improve specificity of AAV9 mediated transgene expression following intra-vascular gene delivery in neonatal mice.

Pacak CA, Sakai Y, Thattaliyath BD, Mah CS, Byrne BJ.

Genet Vaccines Ther. 2008 Sep 23;6:13. doi: 10.1186/1479-0556-6-13.

31.
32.

Long-term skeletal muscle protection after gene transfer in a mouse model of LGMD-2D.

Pacak CA, Walter GA, Gaidosh G, Bryant N, Lewis MA, Germain S, Mah CS, Campbell KP, Byrne BJ.

Mol Ther. 2007 Oct;15(10):1775-81. Epub 2007 Jul 24.

33.

Physiological correction of Pompe disease by systemic delivery of adeno-associated virus serotype 1 vectors.

Mah C, Pacak CA, Cresawn KO, Deruisseau LR, Germain S, Lewis MA, Cloutier DA, Fuller DD, Byrne BJ.

Mol Ther. 2007 Mar;15(3):501-7. Epub 2007 Jan 23.

34.

Recombinant adeno-associated virus serotype 9 leads to preferential cardiac transduction in vivo.

Pacak CA, Mah CS, Thattaliyath BD, Conlon TJ, Lewis MA, Cloutier DE, Zolotukhin I, Tarantal AF, Byrne BJ.

Circ Res. 2006 Aug 18;99(4):e3-9. Epub 2006 Jul 27.

PMID:
16873720
35.

Sustained correction of glycogen storage disease type II using adeno-associated virus serotype 1 vectors.

Mah C, Cresawn KO, Fraites TJ Jr, Pacak CA, Lewis MA, Zolotukhin I, Byrne BJ.

Gene Ther. 2005 Sep;12(18):1405-9.

PMID:
15920463
36.

A targeted gain of function screen in the embryonic CNS of Drosophila.

McGovern VL, Pacak CA, Sewell ST, Turski ML, Seeger MA.

Mech Dev. 2003 Oct;120(10):1193-207.

37.

Genetic fate of recombinant adeno-associated virus vector genomes in muscle.

Schnepp BC, Clark KR, Klemanski DL, Pacak CA, Johnson PR.

J Virol. 2003 Mar;77(6):3495-504.

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