Polyglutamine (polyQ) diseases are a family of dominantly transmitted neurodegenerative disorders caused by an abnormal expansion of CAG trinucleotide repeats in the protein-coding regions of the respective disease-causing genes. Despite their simple genetic basis, the etiology of these diseases is far from clear. Over the past two decades, Drosophila has proven to be successful in modeling this family of neurodegenerative disorders, including the faithful recapitulation of pathological features such as polyQ length-dependent formation of protein aggregates and progressive neuronal degeneration. Additionally, it has been valuable in probing the pathogenic mechanisms, in identifying and evaluating disease modifiers, and in helping elucidate the normal functions of disease-causing genes. Knowledge learned from this simple invertebrate organism has had a large impact on our understanding of these devastating brain diseases.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4644473 | PMC |
| http://dx.doi.org/10.1016/j.expneurol.2015.08.002 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
A tribute to Laszlo Zaborszky: pioneering discoveries in the basal forebrain and inspiring generations of neuroscientists.
Brain Struct Funct
December 2024
School of Medicine, Department of Neuropharmacology, Western Sydney University, Locked Bag 1797, Penrith, NSW, 2751, Australia.
This editorial celebrates the 80th birthday of Distinguished Professor Laszlo Zaborszky, co-founder of Brain Structure and Function, and reflects on his monumental contributions to neuroscience, particularly his pioneering work on the cholinergic basal forebrain. Professor Zaborszky's research has reshaped our understanding of this brain region's organization and function, uncovering its critical role in cognitive processes such as learning, memory, and attention. His findings have challenged longstanding assumptions, demonstrating that the cholinergic projections to the cortex are highly organized, with implications for neurodegenerative diseases like Alzheimer's.
View Article and Find Full Text PDFExploring fatty acid metabolism in Alzheimer's disease: the key role of CPT1A.
Sci Rep
December 2024
Department of Neurology, Union Hospital of Jilin University, Changchun, 130000, China.
Alzheimer's disease (AD) is a severe neurodegenerative disease, and the most common type of dementia, with symptoms of progressive cognitive dysfunction and behavioral impairment. Studying the pathogenesis of AD and exploring new targets for the prevention and treatment of AD is a very worthwhile challenge. Accumulating evidence has highlighted the effects of fatty acid metabolism on AD.
View Article and Find Full Text PDFBrd4 modulates metabolic endotoxemia-induced inflammation by regulating colonic macrophage infiltration in high-fat diet-fed mice.
Commun Biol
December 2024
Fujian Key Laboratory of Translational Research in Cancer and Neurodegenerative Diseases, Institute for Basic Medical Sciences, School of Basic Medical Sciences, Fujian Medical University, Fuzhou, China.
High-fat diet (HFD) induces low-grade chronic inflammation, contributing to obesity and insulin resistance. However, the precise mechanisms triggering obesity-associated metabolic inflammation remain elusive. In this study, we identified epigenetic factor Brd4 as a key player in this process by regulating the expression of Ccr2/Ccr5 in colonic macrophage.
View Article and Find Full Text PDFStructural MRI study of Pareidolia and Visual Hallucinations in Drug-Naïve Parkinson's disease.
Sci Rep
December 2024
Department of Neurology, The Jikei University School of Medicine, 3-25-8 Nishi-Shimbashi, Minato-ku, Tokyo, 105-8461, Japan.
Visual hallucinations (VH) and pareidolia, a type of minor hallucination, share common underlying mechanisms. However, the similarities and differences in their brain regions remain poorly understood in Parkinson's disease (PD). A total of 104 drug-naïve PD patients underwent structural MRI and were assessed for pareidolia using the Noise Pareidolia Test (NPT) were enrolled.
View Article and Find Full Text PDFExpanded and independent validation of the NoMoFA scale for Parkinson's disease: the Italian version.
J Neurol
December 2024
Department of Neurosciences Rita Levi Montalcini, University of Turin, Turin, Italy.
Introduction: Non-motor symptoms (NMS) in Parkinson's disease (PD) can fluctuate daily, impacting patient quality of life. The Non-Motor Fluctuation Assessment (NoMoFA) Questionnaire, a recently validated tool, quantifies NMS fluctuations during ON- and OFF-medication states. Our study aimed to validate the Italian version of NoMoFA, comparing its results to the original validation and further exploring its clinimetric properties.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!