Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8504386 | PMC |
| http://dx.doi.org/10.4103/1673-5374.320986 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Liver matrin-3 protects mice against hepatic steatosis and stress response via constitutive androstane receptor.
Mol Metab
August 2024
Department of Biochemistry, University of Nebraska - Lincoln, Beadle Center, 1901 Vine St, Lincoln, NE 68588, USA; Nebraska Center for Integrated Biomolecular Communication (NCIBC), University of Nebraska - Lincoln, Lincoln, NE 68588, USA; Nebraska Center for the Prevention of Obesity Diseases through Dietary Molecules, University of Nebraska - Lincoln, USA. Electronic address:
Article Synopsis
- Researchers are studying a liver disease called MASLD, which is getting more common because many people are becoming overweight, and they want to find better treatments.
- They looked at a protein called Matrin-3 that helps control how genes work in the liver and its role in preventing fat buildup and stress responses.
- When Matrin-3 was removed from certain mice, it made their livers worse when they ate a high-fat diet, showing that Matrin-3 is important for keeping liver health by helping a special receptor called CAR function properly.
The role of Matrin-3 in physiology and its dysregulation in disease.
Biochem Soc Trans
June 2024
Department of Chemistry, Washington University, St. Louis, MO 63130, U.S.A.
The dysfunction of many RNA-binding proteins (RBPs) that are heavily disordered, including TDP-43 and FUS, are implicated in amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD). These proteins serve many important roles in the cell, and their capacity to form biomolecular condensates (BMCs) is key to their function, but also a vulnerability that can lead to misregulation and disease. Matrin-3 (MATR3) is an intrinsically disordered RBP implicated both genetically and pathologically in ALS/FTD, though it is relatively understudied as compared with TDP-43 and FUS.
View Article and Find Full Text PDFNeuronal activity regulates Matrin 3 abundance and function in a calcium-dependent manner through calpain-mediated cleavage and calmodulin binding.
Proc Natl Acad Sci U S A
April 2023
Neuroscience Graduate Program, University of Michigan, Ann Arbor, MI 48109.
RNA-binding protein (RBP) dysfunction is a fundamental hallmark of amyotrophic lateral sclerosis (ALS) and related neuromuscular disorders. Abnormal neuronal excitability is also a conserved feature in ALS patients and disease models, yet little is known about how activity-dependent processes regulate RBP levels and functions. Mutations in the gene encoding the RBP Matrin 3 (MATR3) cause familial disease, and MATR3 pathology has also been observed in sporadic ALS, suggesting a key role for MATR3 in disease pathogenesis.
View Article and Find Full Text PDFMatrin-3 dysfunction in myopathy and motor neuron degeneration.
Neural Regen Res
March 2022
Department of Pediatrics, Division of Child Neurology; Department of Human Genetics, School of Public Health, University of Pittsburgh; Center for Neuroscience Institute, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Pathomechanisms of ALS8: altered autophagy and defective RNA binding protein (RBP) homeostasis due to the VAPB P56S mutation.
Cell Death Dis
May 2021
Institute of Neuropathology, RWTH Aachen University Medical School, Pauwelsstr. 30, 52074, Aachen, Germany.
Mutations in RNA binding proteins (RBPs) and in genes regulating autophagy are frequent causes of familial amyotrophic lateral sclerosis (fALS). The P56S mutation in vesicle-associated membrane protein-associated protein B (VAPB) leads to fALS (ALS8) and spinal muscular atrophy (SMA). While VAPB is primarily involved in the unfolded protein response (UPR), vesicular trafficking and in initial steps of the autophagy pathway, the effect of mutant P56S-VAPB on autophagy regulation in connection with RBP homeostasis has not been explored yet.
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!