Brain: Research reveals the genetic mechanisms of neurodegenerative diseases.

16 July 2020 /--- About 50 million people worldwide have dementiaThe two most common forms are Alzheimer's disease and prefrontal lobe degeneration (FTLD), which cause sepsis to cause neurons in specific parts of the brain to stop functioning, triggering memory loss and other behavioural or personality changesThe World Health Organization predicts that without a cure, that number could increase by 10 million a yearHowever, predicting the onset of these diseases can be challenging because neurodegeneration can begin years before people develop any external symptomsIn a recent study published in the journal Brain, researchers at Cornell University revealed the role of the risk factor TMEM106B in myelin formation and the onset of Alzheimer's diseaseFenghua Hu, associate professor in the Department of Molecular Biology and Genetics, is the lead author of this paper"I want to have a better understanding of the molecular and cellular mechanisms of neurodegeneration," Hu saidIt is hoped that our research will promote the therapeutic development of treatment options for patients with neurodegenerative diseases and other brain diseasesHer team first studied a specific gene called TMEM106B, which has previously been identified as a risk factor for a variety of neurodegenerative diseases, including Alzheimer's disease and FTLDThe researchers also know that specific mutations in the gene can cause neurological defects, known as hypomyelocyte malnutrition, which leads to a lack of myelin in the brain, leading to a decline in motor skills and intelligenceMyelin is the adipose tissue that wraps nerve fibers or axons in the nervous systemThis tissue forms a sheath around nerve fibers, protecting them from damage and allowing electrical currents to pass quickly along the nervesHu et alwant to know exactly how a mutation in TMEM106B can do so much damageShe would also like more information on how the gene regulates myelin formation and maintenance under normal circumstancesSince Hu's lab saw The expression of TMEM106B in the protrusion cells responsible for forming protective myelin tissueTMEM106B is located in a lysosomeLike the stomach, lysozymes must maintain a specific pH to maintain the activity of their enzymesWhen fewer protrusion cells form myelin, the lysozyme experience removes all unrelated substancesThey can also store the main membrane proteins of myelin and deposit them in the area around the nerve fibersHu's team found that the TMEM106B mutation blocked the movement of the gene to regulate the pH inside the lysozyme and the lysozyme itself, thereby inhibiting the ability of fewer protrusion cells to build a tight myelin layerHu also used mouse models to note that the overall lack of TMEM106B caused abnormal lysosome movement in the protrusion cells, resulting in defects in myelinIn addition, the team observed changes in behavior in mutant mice, including poor motor coordination(Bioon.com) Source: Gene yields insights into the causes of the original source of the neurodegeneration: Tuancheng Feng et alA role of the frontotemporal lobar degeneration risk factor TMEM106B in myelination, Brain (2020)DOI: 10.1093/brain/awaa154.