BPAN 101: MOVEMENT DISORDERS
WHAT IS DYSTONIA? AND HOW IS IT RELATED TO BPAN?
Autophagy, derived from the Greek "auto" (self) and "phaegin" (to eat) is a
biological process whereby cells continually renew, recycle and eliminate waste products which plays a key role in your body’s ability to detoxify, repair, and regenerate itself to achieve cellular homeostasis. This internal balance is achieved through the vital process of autophagy where unwanted, unused materials, such as aggregate proteins and damaged cell components are disposed of through the controlled digestion of internal cell components. It is also an important component in cellular defense against pathogenic microorganisms and viral infection. Autophagy is crucial for maintaining neuron homeostasis and plays a complex physiological and pathophysiological role in many neurogenerative diseases and cancers.
WHAT IS PARKINSONS? AND HOW IS IT RELATED TO BPAN?
Autophagy is a multi-step process, which includes sequestration, whereby defective cell components (waste) are wrapped and sealed by a membrane called an autophagosome. These vesicular structures, then transport the waste to lysosomes, within our cells were enzymes break down the unwanted cellular material. From this "recyling" process, small molecules of usable material is produced. Although essential for the proper functioning of the body, the role of autophagy in disease is highly complex. Autophagy defends the body against pathogens, viral infection and foreign invaders, however—cancer cells exploit autophagy to survive in nutrient-poor tumors and to increase growth and aggressiveness.
POTENTIAL FOR A CURE THROUGH PARKINSONS RESEARCH?
Autophagy plays an essential role in assisting cells maintain normal function and survive under internal and external environmental stressors. Research suggests that WDR45 helps to maintain neural homeostasis through its role in autophagy. BPAN patients have shown lower autophagic activity and have also exhibited atypical autophagic structures. Identifying how and at what specific stages of the autophagy WDR45 affects the autophagic process, is an area of research which may help scientists better understand the disease and further potential therapeutic treatments.
LEARN MORE ABOUT SCIENTIFIC RESEARCH RELATED TO DYSTONIA AND PARKINSONS:
Wang Y, Shi Y, Wei H.
J Alzheimers Dis Parkinsonism. 2017 Aug;7(5). pii: 374. doi: 10.4172/2161-0460.1000374. Epub 2017 Sep 15.
Zhao YG, Sun L, Miao G, Ji C, Zhao H, Sun H, Miao L, Yoshii SR, Mizushima N, Wang X, Zhang H.
Autophagy. 2015;11(6):881-90. doi: 10.1080/15548627.2015.1047127.