I wanted to talk about a neurodegenerative disease that we didn't discuss in class. If anyone watches/watched House- 13 (Olivia Wilde's character) was genetically predispositioned for Huntington's Disease because her mother died from it.
Huntington's is a neurodegenerative disorder characterized by both psychiatric, physical and cognitive impairment. It can be diagnosed early in life for those with genetic history of the disease as it features a repeating -CAG- pattern on the huntington gene, resulting in misfolded proteins.
Reactive oxygen species play a big role in neurodegenerative diseases and the inflammatory response, as we have learned in class. Antioxidants work by scavenging the free radicals, essentially blocking them from causing damage. This study looks at a whole bunch of antioxidants and how they could be potential treatment for slowing the progression of HD.
http://www.ncbi.nlm.nih.gov/pubmed/22138129. You can find the full article by typing "Antioxidants in Huntington's Disease" into the Library journal search and clicking out to electronic sources.
I really liked how well written this article is. Everything from what HD is to its abnormal genetic coding, was basically written out for you. I embarrassingly enjoyed figure 1 and table 1. Figure one because the diagram was easy to follow how ROS affects the Mitochondria with the MHTT fragment to decrease the production of PGC-1alpha, leading to less peroxisome enyzmes. One thing I didn't really understand is HTT, I understand it is expressed in the inner cytoplasmic membrane wall, but why is it important? How is the HTT gene crucial to human life? Also, I wonder if they have any other studys on how a mutant HTT gene can worsen or progress HD based its production of fragments. I think understanding the process of MHTT fragments can help researchers discover much more and maybe solve the problem. Table 1 was a nice refresher on the different types of anti-oxidants and there main affects. This is important because the article has strong opinion on ROS really causing damage on the other mitochodria. I think the website Livestrong provides a small summary of HD and the foods that can help with ROS production, agreeing with the article.
ReplyDeletehttp://www.livestrong.com/article/288459-antioxidants-that-could-help-huntingtons-disease-patients/
I was interested in the role of the HTT gene, as well, and decided to do a little research to see its role in a healthy individual. The HTT gene codes for a protein called Huntington, whose exact biological function still has not been elucidated. One paper claims Huntington causes the up-regulation of brain-derived neurotrophic factor (BDNF), which is an anti-apoptotic neurotrophin that is essential for neuronal survival. If this is in fact the case, it is clear how a mutation in this protein can cause detrimental effects in the nervous system. Still more evidence has implicated Huntington in various other biological pathways, so clearly more research needs to be done to determine the protein's full impact.
DeleteHere is the paper about Huntington's role in the production of BDNF:
http://www.ncbi.nlm.nih.gov/pubmed/11408619
Wow dude that was a really nice article.
Delete