Pedro Gonzalez-Alegre M.D.

GonzalezP
Assistant Professor.
Neurology
Research area(s): 
Neurological Disorders
(319) 335-7498
FAX: 
(319) 335-9517
3111A
MERF
Lab phone: 
(319) 335-9178
3111
MERF
Research: 

The major goal of my laboratory is to understand the biological basis of neurological disorders caused by dysfunction of the basal ganglia and to develop novel therapies for this group of neurological diseases. Currently, my studies focus on a disease known as DYT1 or Oppenheim\'s dystonia, the most common form of inherited dystonia. DYT1 dystonia, a dominantly inherited, incurable disease, is caused by a three-nucleotide deletion in the gene TOR1A that causes the loss of a glutamic acid in the protein torsinA. TorsinA is a AAA protein (ATPases Associated with diverse cellular Activities) that resides primarily in the endoplasmic reticulum. However, torsinA carrying the disease-causing mutation accumulates in the nuclear envelope generating cytoplasmic membranous inclusions known as spheroid bodies. Analyses of dominant negative mutants suggest that torsinA normally functions within the perinuclear space of the nuclear envelope.