Depletion of p62 reduces nuclear inclusions and paradoxically ameliorates disease phenotypes in Huntington's model mice
نویسندگان
چکیده
منابع مشابه
Depletion of p62 reduces nuclear inclusions and paradoxically ameliorates disease phenotypes in Huntington's model mice.
Huntington's disease (HD) is a dominantly inherited genetic disease caused by mutant huntingtin (htt) protein with expanded polyglutamine (polyQ) tracts. A neuropathological hallmark of HD is the presence of neuronal inclusions of mutant htt. p62 is an important regulatory protein in selective autophagy, a process by which aggregated proteins are degraded, and it is associated with several neur...
متن کاملSialyllactose ameliorates myopathic phenotypes in symptomatic GNE myopathy model mice.
Patients with GNE myopathy, a progressive and debilitating disease caused by a genetic defect in sialic acid biosynthesis, rely on supportive care and eventually become wheelchair-bound. To elucidate whether GNE myopathy is treatable at a progressive stage of the disease, we examined the efficacy of sialic acid supplementation on symptomatic old GNE myopathy mice that have ongoing, active muscl...
متن کاملDepletion of coagulation factor XII ameliorates brain pathology and cognitive impairment in Alzheimer disease mice.
Vascular abnormalities and inflammation are found in many Alzheimer disease (AD) patients, but whether these changes play a causative role in AD is not clear. The factor XII (FXII) -initiated contact system can trigger both vascular pathology and inflammation and is activated in AD patients and AD mice. We have investigated the role of the contact system in AD pathogenesis. Cleavage of high-mol...
متن کاملFiltered Kombucha tea ameliorates the leaky gut syndrome in young and old mice model of colitis
Objective(s): Zonula occludens proteins (ZO-1 and ZO-2) are important intracellular tight junction (TJ)-associated proteins that link the cell cytoskeleton to the trans-membrane TJ proteins. Destruction of TJ proteins is called the “leaky gut syndrome” and has been observed in some of the gastrointestinal diseases such as the inflammatory bowel disease (IBD). So, ther...
متن کاملVarenicline Ameliorates Learning and Memory Deficits in Amyloid β(25–35) Rat Model of Alzheimer’s Disease
Introduction: Alzheimer’s disease (AD) is a enfeeble neurodegenerative disorder characterized by increased β-amyloid (Aβ) deposition and neuronal dysfunction leading to impaired learning and recall. Among proposed risk factors, impaired cholinergic transmission is a main cause for incidence of disease. Methods: In the present study, effects of the intracerebroventricularly administration of an ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Human Molecular Genetics
سال: 2014
ISSN: 1460-2083,0964-6906
DOI: 10.1093/hmg/ddu522