A5084752116- Mitochondrial Function and Pathology
- Genetic Neurodegenerative Diseases
- Adipose Tissue and Metabolism
- Muscle Physiology and Disorders
- Alzheimer's disease research and treatments
- Metabolism and Genetic Disorders
- Amyotrophic Lateral Sclerosis Research
- Receptor Mechanisms and Signaling
- Neurological disorders and treatments
- Neuroscience and Neuropharmacology Research
- Nuclear Receptors and Signaling
- Nerve injury and regeneration
- COVID-19 Clinical Research Studies
- Parkinson's Disease Mechanisms and Treatments
- Long-Term Effects of COVID-19
- Tryptophan and brain disorders
- Neurological diseases and metabolism
- Diet and metabolism studies
- COVID-19 and Mental Health
- Nicotinic Acetylcholine Receptors Study
- Neurogenetic and Muscular Disorders Research
Cornell University
2010-2021
Weill Cornell Medicine
2021
MIND Research Institute
2016-2021
New York Hospital Queens
2011-2014
NewYork–Presbyterian Hospital
2011-2014
Presbyterian Hospital
2011-2013
There is substantial evidence that impairment of peroxisome proliferator-activated receptor (PPAR)-γ-coactivator 1α (PGC-1α) levels and activity play an important role in Huntington's disease (HD) pathogenesis. We tested whether pharmacologic treatment with the pan-PPAR agonist bezafibrate would correct a deficiency PGC-1α exert beneficial effects transgenic mouse model HD. found administration diet restored PGC-1α, PPARs downstream genes to which occur wild-type mice. were significant...
Huntington's disease (HD) is an incurable neurological disorder caused by abnormal glutamine repeat expansion in the huntingtin (Htt) protein. In present studies, we investigated role of Transducers Regulated cAMP response element-binding (CREB) protein activity (TORCs) HD, since TORCs play important expression transcriptional co-regulator peroxisome proliferator-activated receptor gamma coactivator 1α (PGC-1α), whose impaired HD. We found significantly decreased TORC1 levels STHdhQ111 cells...
Parkinson's disease (PD) is a neurodegenerative in which the etiology of 90 percent patients unknown. Pesticide exposure major risk factor for PD, and paraquat (PQ), pyridaben (PY) maneb (MN) are amongst most widely used pesticides. We studied mRNA expression using transcriptome sequencing (RNA-Seq) ventral midbrain (VMB) striatum (STR) PQ, PY paraquat+maneb (MNPQ) treated mice, followed by pathway analysis. found concordance signaling pathways between three pesticide models both VMB STR as...
Huntington's disease (HD) is a devastating illness and at present there no modifying therapy or cure for it; management of the limited to few treatment options amelioration symptoms. Recently, we showed that administration bezafibrate, pan-PPAR agonist, increases expression PGC-1α mitochondrial biogenesis, improves phenotype survival in R6/2 transgenic mouse model HD. Since mice represent 'truncated' huntingtin (Htt) HD, tested efficacy bezafibrate 'full-length' Htt model, BACHD mice....
<i>Background:</i> Reduced peroxisome proliferator-activated receptor-γ coactivator 1α (PGC1α) gene expression has been observed in striatal cell lines, transgenic mouse models of Huntington’s disease (HD), and brain tissue from HD patients. As this protein is a key transcription regulator the many mitochondrial proteins, these observations strongly support role aberrant function pathogenesis HD. The PGC1α undergoes posttranslational modifications that affect its transcriptional...