Mutations that disrupt the function of the DNA/RNA-binding protein FUS could cause amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. One of the key features in ALS pathogenesis is the formation of insoluble protein aggregates containing aberrant isoforms of the FUS protein in the cytoplasm of upper and lower motor neurons. Reproduction of human pathology in animal models is the main tool for studying FUS-associated pathology and searching for potential therapeutic agents for ALS treatment. In this review, we provide a systematic analysis of the role of FUS protein in ALS pathogenesis and an overview of the results of modelling FUS-proteinopathy in animals.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1134/S0006297924140037 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
An integrative taxonomic approach reveals two putatively novel species of phlebotomine sand fly (Diptera: Psychodidae) in Thailand.
Parasit Vectors
January 2025
Center of Excellence in Veterinary Parasitology, Department of Pathology, Faculty of Veterinary Science, Chulalongkorn University, Bangkok, 10330, Thailand.
Background: The subfamily Phlebotominae comprises 1028 species of sand fly, of which only 90 are recognized as vectors of pathogenic agents such as Trypanosoma, Leishmania, and Bartonella. In Thailand, leishmaniasis-a sand fly-borne disease-is currently endemic, with 36 documented sand fly species. However, many cryptic species likely remain unidentified.
View Article and Find Full Text PDFEvaluation of glomerular filtration rate estimation through plasma clearance of iohexol in seagulls (Larus michahellis).
BMC Vet Res
January 2025
Department of Veterinary Sciences, Veterinary Teaching Hospital "Mario Modenato", University of Pisa, via Livornese snc, San Piero a Grado, Pisa, Italy.
Background: Current endogenous indicators utilised in avian medicine are not sensitive enough to detect renal disease in its early stages. Alternative markers ought to be examined as a result. The aim is to investigate the accuracy of limited-sampling models for glomerular filtration rate (GFR) in adult seagulls using plasma clearance of iohexol (IOX).
View Article and Find Full Text PDFThe effects of runs-of-homozygosity on pig domestication and breeding.
BMC Genomics
January 2025
Key Laboratory of Genetic Evolution & Animal Models and Yunnan Key Laboratory of Molecular Biology of Domestic Animals, Kunming Institute of Zoology, Chinese Academy of Sciences, Kunming, 650223, China.
Background: Since their domestication, recent inbreeding together with intensive artificial selection and population bottlenecks have allowed the prevalence of deleterious mutations and the increase of runs-of-homozygosity (ROH) in domestic pigs. This makes pigs a good model to understand the genetic underpinnings of inbreeding depression.
Results: Here we integrated a comprehensive dataset comprising 7239 domesticated pigs and wild boars genotyped by single nucleotide polymorphism (SNP) chips, along with phenotypic data encompassing growth, reproduction and disease-associated traits.
TAX1BP1 regulates the apoptosis of renal tubular epithelial cells in ischemia/reperfusion injury via the NF-kB/PMAIP1 signaling pathway.
Inflamm Res
January 2025
Department of Nephrology, the Third Affiliated Hospital of Chongqing Medical University, Chongqing, China.
Background: The pathogenesis of acute kidney injury (AKI) is not fully understood. Tax1-binding protein 1 (TAX1BP1) modulates inflammation and apoptosis through the NF-kB signaling pathway, however, its specific role in ischemic AKI remains unclear.
Methods: We injected a TAX1BP1 overexpression plasmid into the tail vein of male C57BL/6 mice, followed by clamping the bilateral renal arteries to induce AKI.
Preeclampsia (PE) is a prevalent and severe pregnancy complication that significantly impacts maternal and perinatal health. Epidemiological studies and animal experiments have demonstrated that PE adversely affects the cardiovascular and nervous systems of offspring, increasing their risk of hypertension and renal pathology. However, the mechanisms underlying this increased risk remain unclear.
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!