Regulation of CuZnSOD and Its Redox Signaling Potential: Implications for Amyotrophic Lateral Sclerosis
نویسندگان
چکیده
منابع مشابه
Misfolded CuZnSOD and amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of motor neurons. The inherited form of the disease, familial ALS, represents 5-10% of the total cases, and the best documented of these are due to lesions in SOD1, the gene encoding copper-zinc superoxide dismutase (CuZnSOD). The mechanism by which mutations in SOD1 cause familial ALS is currently unknown. Two hypotheses...
متن کاملRedox Regulation in Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that results from the death of upper and lower motor neurons. Due to a lack of effective treatment, it is imperative to understand the underlying mechanisms and processes involved in disease progression. Regulations in cellular reduction/oxidation (redox) processes are being increasingly implicated in disease. Here we discuss th...
متن کاملRetinoid signaling alterations in amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disease for which effective therapeutic interventions and an understanding of underlying disease mechanism are lacking. A variety of biochemical pathways are believed to contribute to the pathophysiology of ALS that are common to both sporadic and familial forms of the disease. Evidence from both human and animal studies indicates tha...
متن کاملRedox modifier genes and pathways in amyotrophic lateral sclerosis.
Enhanced redox-stress caused by neuroinflammation, mitochondria, and NADPH oxidases has been hypothesized to play critical roles in disease progression of amyotrophic lateral sclerosis (ALS). However, distinguishing whether the redox-stress observed in ALS is due to a primary defect in cellular reactive oxygen species metabolism/catabolism, or is a secondary consequence of neuroinflammation, ha...
متن کاملPotential Environmental Factors in Amyotrophic Lateral Sclerosis.
The causes of amyotrophic lateral sclerosis (ALS) are largely unknown, and may always be multiple, including environmental factors. Monogenetic determinants of ALS are involved in roughly 20% of all cases (including 10% familial cases). Less well understood multigenetic causes may contribute to another 20% to 80%. Environmental factors likely play a role in the development of ALS in susceptible...
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: Antioxidants & Redox Signaling
سال: 2014
ISSN: 1523-0864,1557-7716
DOI: 10.1089/ars.2013.5385