ON THE LYSOSOMAL DEGRADATION OF NEUROFIBROMIN AND ITS PHOSPHORYLATION IN CULTURED MELANOCYTES

摘要: Neurofibromatosis type 1 (NF1) is one of the most common inherited disorders in humans. Most NF1 gene mutations result a reduction amount neurofibromin to about 50%. Recently, we found that level can be regulated post-translationally through alteration its half-life. Here, investigated whether lysosomes are involved this post-translational regulation cultured melanocytes patients and controls. When lysosomal degradation was inhibited by chloroquine, an increase factor 2 3, correlating with increased half-life, measured. Incubation phosphoprotein-phosphatase inhibitors also content melanocytes. Investigations on phosphorylation revealed basal growth factor-deprived medium upon incubation stimulators PMA or bFGF. Because both factors able half-life neurofibromin, suggest important step protecting against specific degradation.