Misfolded proteins in Huntington disease fetal grafts: further evidence of cell-to-cell transfer?
نویسندگان
چکیده
Hypomyelinating leukodystrophies: Translational research progress and prospects. pg. 5-19 Pouwels, Petra J. W. PhD 1; Vanderver, Adeline MD 2; Bernard, Genevieve MD, MSc, FRCPC 3; Wolf, Nicole I. MD, PhD 4; Dreha-Kulczewksi, Steffi F. MD 5; Deoni, Sean C. L. PhD 6; Bertini, Enrico MD, PhD 7; Kohlschutter, Alfried MD 8; Richardson, William FMedSci, FRS 9; ffrench-Constant, Charles PhD 9; Kohler, Wolfgang MD 10; Rowitch, David MD, PhD 11; Barkovich, James A. MD 12,13
منابع مشابه
The Seeds of Neurodegeneration: Prion-like Spreading in ALS
Misfolded proteins accumulating in several neurodegenerative diseases (including Alzheimer, Parkinson, and Huntington diseases) can cause aggregation of their native counterparts through a mechanism similar to the infectious prion protein's induction of a pathogenic conformation onto its cellular isoform. Evidence for such a prion-like mechanism has now spread to the main misfolded proteins, SO...
متن کاملCan regional spreading of amyotrophic lateral sclerosis motor symptoms be explained by prion-like propagation?
Progressive accumulation of specific misfolded protein is a defining feature of amyotrophic lateral sclerosis (ALS), similarly seen in Alzheimer disease, Parkinson disease, Huntington disease and Creutzfeldt-Jakob disease. The intercellular transfer of inclusions made of tau, α-synuclein and huntingtin has been demonstrated, revealing the existence of mechanisms reminiscent of those by which pr...
متن کاملThe Effect of Normobaric Hyperoxia on Superoxide Dismutase Activity and Neurologic Deficits in Huntington Animal Model
Introduction: resent studies have been shown that normobaric hyperoxia (HO) can induce excitotoxicity and stress oxidative tolerance (ETT) in variety of organs such as brain. In this study, we examined the intermittent dose of normobaric hyperoxia (HO) on neurologic deficit, and superoxide dismutase activity in brain tissue of Huntington animal model. Method: The rats were divided to three...
متن کاملConformational Analysis of Misfolded Protein Aggregation by FRET and Live-Cell Imaging Techniques
Cellular homeostasis is maintained by several types of protein machinery, including molecular chaperones and proteolysis systems. Dysregulation of the proteome disrupts homeostasis in cells, tissues, and the organism as a whole, and has been hypothesized to cause neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS) and Huntington's disease (HD). A hallmark of neurodegenera...
متن کاملChanges in BiP availability reveal hypersensitivity to acute endoplasmic reticulum stress in cells expressing mutant huntingtin.
Huntington's disease (HD) is caused by expanded glutamine repeats within the huntingtin (Htt) protein. Mutant Htt (mHtt) in the cytoplasm has been linked to induction of the luminal endoplasmic reticulum (ER) stress pathway, the unfolded protein response (UPR). How mHtt impacts the susceptibility of the ER lumen to stress remains poorly understood. To investigate molecular differences in the ER...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Annals of neurology
دوره 76 1 شماره
صفحات -
تاریخ انتشار 2014