[HTML][HTML] Phenylbutyrate up-regulates the DJ-1 protein and protects neurons in cell culture and in animal models of Parkinson disease
W Zhou, K Bercury, J Cummiskey, N Luong… - Journal of Biological …, 2011 - ASBMB
Parkinson disease is caused by the death of midbrain dopamine neurons from oxidative
stress, abnormal protein aggregation, and genetic predisposition. In 2003, Bonifati et al.(23)
found that a single amino acid mutation in the DJ-1 protein was associated with early-onset,
autosomal recessive Parkinson disease (PARK7). The mutation L166P prevents
dimerization that is essential for the antioxidant and gene regulatory activity of the DJ-1
protein. Because low levels of DJ-1 cause Parkinson, we reasoned that overexpression …
stress, abnormal protein aggregation, and genetic predisposition. In 2003, Bonifati et al.(23)
found that a single amino acid mutation in the DJ-1 protein was associated with early-onset,
autosomal recessive Parkinson disease (PARK7). The mutation L166P prevents
dimerization that is essential for the antioxidant and gene regulatory activity of the DJ-1
protein. Because low levels of DJ-1 cause Parkinson, we reasoned that overexpression …