protein degradation process, will be discussed along with the Ubiquitin Ligase, a major player in the mechanism. Ubiquitination is a post-translational modification where ubiquitin, an 8kDa protein that is highly conserved in eukaryotes, is attached to a substrate protein (Lu et al., 2008). Ubiquitin is attached to a targeted protein through a sequential cascade which involves E1 activating, E2 conjugating, and E3 ligase enzymes. This ubiquitin tagging is necessary for many cellular processes, including
MUTANT HUNTINGTIN PROTEIN AGGREGATION AND PROTEASOMAL DISFUNCTION AN INSILICO STUDY 1. INTRODUCTION 1.1 Huntingtons Disease Huntington’s disease (HD) is a progressive autosomal dominant neurodegenerative genetic disorder. HD was originally named Huntington’s chorea after Dr.George Huntington, an American physician who first gave a detailed note on the symptoms and course of the disease in 1872.Recently the name has been changed to Huntington’s disease to emphasize the fact that chorea is not the
1. Histone modifications can effect transcription by altering the chromatin structure or the interaction with other regulatory proteins. Addition of positive or negative charges through the modifications disrupts the electrostatic interaction between the histones and DNA, which modulates the chromatin structure and therefore, the accessibility of DNA to the regulatory proteins. DNA methylation is an epigenetic modification which can influence the interaction between transcription factors and CpG
Understanding Huntington's Disease Huntington's disease is an inherited neurodegenerative disorder. It is passed on to children from one or both parents (though two parents with Huntington's is extraordinarily rare) in an autosomal dominant manner. This is different from autosomal recessive disorder, which requires two altered genes (one from each parent) to inherit the disorder. So if one parent has it, and passes the gene on to a child, that child will develop Huntington's disease if they live