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Sylvette Ayala-Peña
Title Principal Investigator
Faculty Rank Associate Professor
Degree Ph.D.
Institution University of Puerto Rico
Department Pharmacology and Toxicology
Clusters Neurological Disorders and Mental Health
Obesity and Metabolic Syndromes
Address
University of Puerto Rico
Medical Sciences Campus
P.O. Box 365067
City San Juan
State PR
Postal Code 00936-5067
Telephone (787) 758-2525
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  Huntington’s Disease (HD) is a neurodegenerative disorder for which no treatment has been identified to prevent, limit, or cure symptoms of involuntary choreiform movements, behavioral abnormalities, dementia, and motor dysfunction as a result of neuronal loss in the striatum and cerebral cortex. Although it is known that HD is caused by mutations in the huntingtin gene, the causality of this mutation in the mechanism of neuronal loss remains unknown. Compelling evidence suggests that increased oxidative stress and mitochondrial dysfunction contribute to the pathophysiology of HD, thus, a better understanding of why mitochondrial dysfunction may arise during HD neurodegeneration could provide the basis for the development of new pharmacological interventions to delay disease onset and progression. We apply cellular, biochemical and molecular approaches using both in vitro and in vivo experimental models of HD. We also test small molecules with antioxidant activity as interventions to prevent onset and/or progression of the disease. Because aging is the main risk factor for chronic diseases and is associated with increased DNA damage and decreased DNA repair capacity, we are also studying the role of mitochondria in diabetes, an age-associated disorder, and testing pharmacological interventions that may protect mtDNA integrity and mitochondrial function.

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1. De Mello WC, Gerena Y, Ayala-Peña S. Angiotensins and Huntington's Disease: A Study on Immortalized Progenitor Striatal Cell Lines. Front Endocrinol (Lausanne). 2017; 8:108.
2. Ballista-Hernández J, Martínez-Ferrer M, Vélez R, Climent C, Sánchez-Vázquez MM, Torres C, Rodríguez-Muñoz A, Ayala-Peña S, Torres-Ramos CA. Mitochondrial DNA Integrity Is Maintained by APE1 in Carcinogen-Induced Colorectal Cancer. Mol Cancer Res. 2017 Jul; 15(7):831-841.
3. Padín-Irizarry V, Colón-Lorenzo EE, Vega-Rodríguez J, Castro Mdel R, González-Méndez R, Ayala-Peña S, Serrano AE. Glutathione-deficient Plasmodium berghei parasites exhibit growth delay and nuclear DNA damage. Free Radic Biol Med. 2016 06; 95:43-54.
4. Polyzos A, Holt A, Brown C, Cosme C, Wipf P, Gomez-Marin A, Castro MR, Ayala-Peña S, McMurray CT. Mitochondrial targeting of XJB-5-131 attenuates or improves pathophysiology in HdhQ150 animals with well-developed disease phenotypes. Hum Mol Genet. 2016 05 01; 25(9):1792-802.
5. Budworth H, Harris FR, Williams P, Lee DY, Holt A, Pahnke J, Szczesny B, Acevedo-Torres K, Ayala-Peña S, McMurray CT. Suppression of Somatic Expansion Delays the Onset of Pathophysiology in a Mouse Model of Huntington's Disease. PLoS Genet. 2015 Aug; 11(8):e1005267.
6. Escobales N, Nuñez RE, Jang S, Parodi-Rullan R, Ayala-Peña S, Sacher JR, Skoda EM, Wipf P, Frontera W, Javadov S. Mitochondria-targeted ROS scavenger improves post-ischemic recovery of cardiac function and attenuates mitochondrial abnormalities in aged rats. J Mol Cell Cardiol. 2014 Dec; 77:136-46.
7. Jansen RJ, Fonseca-Williams S, Bamlet WR, Ayala-Peña S, Oberg AL, Petersen GM, Torres-Ramos CA. Detection of DNA damage in peripheral blood mononuclear cells from pancreatic cancer patients. Mol Carcinog. 2015 Oct; 54(10):1220-6.
8. López-López L, Nieves-Plaza M, Castro Mdel R, Font YM, Torres-Ramos CA, Vilá LM, Ayala-Peña S. Mitochondrial DNA damage is associated with damage accrual and disease duration in patients with systemic lupus erythematosus. Lupus. 2014 Oct; 23(11):1133-41.
9. Ayala-Peña S. Role of oxidative DNA damage in mitochondrial dysfunction and Huntington's disease pathogenesis. Free Radic Biol Med. 2013 Sep; 62:102-10.
10. Xun Z, Rivera-Sánchez S, Ayala-Peña S, Lim J, Budworth H, Skoda EM, Robbins PD, Niedernhofer LJ, Wipf P, McMurray CT. Targeting of XJB-5-131 to mitochondria suppresses oxidative DNA damage and motor decline in a mouse model of Huntington's disease. Cell Rep. 2012 Nov 29; 2(5):1137-42.
11. Siddiqui A, Rivera-Sánchez S, Castro Mdel R, Acevedo-Torres K, Rane A, Torres-Ramos CA, Nicholls DG, Andersen JK, Ayala-Torres S. Mitochondrial DNA damage is associated with reduced mitochondrial bioenergetics in Huntington's disease. Free Radic Biol Med. 2012 Oct 01; 53(7):1478-88.
12. Castro Mdel R, Suarez E, Kraiselburd E, Isidro A, Paz J, Ferder L, Ayala-Torres S. Aging increases mitochondrial DNA damage and oxidative stress in liver of rhesus monkeys. Exp Gerontol. 2012 Jan; 47(1):29-37.
13. Vogel KS, Perez M, Momand JR, Acevedo-Torres K, Hildreth K, Garcia RA, Torres-Ramos CA, Ayala-Torres S, Prihoda TJ, McMahan CA, Walter CA. Age-related instability in spermatogenic cell nuclear and mitochondrial DNA obtained from Apex1 heterozygous mice. Mol Reprod Dev. 2011 Dec; 78(12):906-19.
14. Acevedo-Torres K, Fonseca-Williams S, Ayala-Torres S, Torres-Ramos CA. Requirement of the Saccharomyces cerevisiae APN1 gene for the repair of mitochondrial DNA alkylation damage. Environ Mol Mutagen. 2009 May; 50(4):317-27.
15. Acevedo-Torres K, Berríos L, Rosario N, Dufault V, Skatchkov S, Eaton MJ, Torres-Ramos CA, Ayala-Torres S. Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's disease. DNA Repair (Amst). 2009 Jan 01; 8(1):126-36.
16. Ayala-Torres S, Chen Y, Svoboda T, Rosenblatt J, Van Houten B. Analysis of gene-specific DNA damage and repair using quantitative polymerase chain reaction. Methods. 2000 Oct; 22(2):135-47.
17. Johnson BH, Russell MJ, Krylov AS, Medh RD, Ayala-Torres S, Regner JL, Thompson EB. Structure-apoptotic potency evaluations of novel sterols using human leukemic cells. Lipids. 2000 Mar; 35(3):305-15.
18. Thompson EB, Medh RD, Zhou F, Ayala-Torres S, Ansari N, Zhang W, Johnson BH. Glucocorticoids, oxysterols, and cAMP with glucocorticoids each cause apoptosis of CEM cells and suppress c-myc. J Steroid Biochem Mol Biol. 1999 Apr-Jun; 69(1-6):453-61.
19. Ayala-Torres S, Zhou F, Thompson EB. Apoptosis induced by oxysterol in CEM cells is associated with negative regulation of c-myc. Exp Cell Res. 1999 Jan 10; 246(1):193-202.
20. Thompson EB, Ayala-Torres S. Oxysterols and apoptosis: evidence for gene regulation outside the cholesterol pathway. Crit Rev Biochem Mol Biol. 1999; 34(1):25-32.
21. Hodgson AV, Ayala-Torres S, Thompson EB, Liehr JG. Estrogen-induced microsatellite DNA alterations are associated with Syrian hamster kidney tumorigenesis. Carcinogenesis. 1998 Dec; 19(12):2169-72.
22. Ayala-Torres S, Moller PC, Johnson BH, Thompson EB. Characteristics of 25-hydroxycholesterol-induced apoptosis in the human leukemic cell line CEM. Exp Cell Res. 1997 Aug 25; 235(1):35-47.
23. Johnson BH, Ayala-Torres S, Chan LN, El-Naghy M, Thompson EB. Glucocorticoid/oxysterol-induced DNA lysis in human leukemic cells. J Steroid Biochem Mol Biol. 1997 Apr; 61(1-2):35-45.
24. Ayala-Torres S, Johnson BH, Thompson EB. Oxysterol sensitive and resistant lymphoid cells: correlation with regulation of cellular nucleic acid binding protein mRNA. J Steroid Biochem Mol Biol. 1994 Mar; 48(4):307-15.

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DNA, Mitochondrial
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