The aim of our laboratory is to understand the molecular pathways that drive protein aggregation and neuronal death in Huntington´s Disease (HD), an inherited neurodegenerative disease characterized by severe motor and cognitive decline. We examine the role of Heat Shock Factor (HSF1), a transcription factor that regulates protein folding, inflammation, and apoptosis. We apply molecular biology, biochemistry, neuroanatomy, and imaging to different HD cellular and mouse models as well as human specimens. Our final goal is to provide new therapeutic strategies to prevent neuronal death and improve ameliorate disease symptoms.
(For a comprehensive list of recent publications, refer to PubMed, a service provided by the National Library of Medicine.)
- Intihar TA, Martinez EA, Gómez-Pastor R. Mitochondrial dysfunction in Huntington's Disease; Interplay between HSF1, p53 and PGC-1α transcription factors. Front Cell Neurosci. 2019 Mar 19;13:103.
- Gómez-Pastor R, Burchfiel ET, Thiele DJ. Regulation of heat shock transcription factors and their roles in physiology and disease. Nat Rev Mol Cell Biol. 2018 Jan;19(1):4-19.
- Gómez-Pastor R, Burchfiel ET, Neef DW, Jaeger AM, Cabiscol E, McKinstry SU, Doss A, Aballay A, Lo DC, Akimov SS, Ross CA, Eroglu C, Thiele DJ. Abnormal degradation of the neuronal stress-protective transcription factor HSF1 in Huntington's disease. Nat Commun. 2017 Feb 13;8:14405.
- Neef DW, Jaeger AM, Gómez-Pastor R, Willmund F, Frydman J, Thiele DJ. A direct regulatory interaction between chaperonin TRiC and stress-responsive transcription factor HSF1. Cell Rep. 2014;9(3):955-966.
- Casani S, Gómez-Pastor R, Matallana E, Paricio N. Antioxidant compound supplementation prevents oxidative damage in a Drosophila model of Parkinson's disease. Free Radic Biol Med. 2013;61:151-160.
- Lavara-Culebras E, Muñoz-Soriano V, Gómez-Pastor R, Matallana E, Paricio N. Effects of pharmacological agents on the lifespan phenotype of Drosophila DJ-1beta mutants. Gene. 2010;462(1-2):26-33.
Current Graduate Students:
Nicole Zarate (Neuroscience, University of Minnesota).