Mitochondrial dysfunction in amyotrophic lateral sclerosis
نویسندگان
چکیده
منابع مشابه
SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease presenting as sporadic (sALS) or familial (fALS) forms. Even if the list of the genes underlining ALS greatly expanded, defects in superoxide dismutase 1 (SOD1), encoding the copper/zinc SOD1, still remain a major cause of fALS and are likely involved also in apparently sporadic presentations. The pathogenesis of ALS is still u...
متن کاملProgressive sudomotor dysfunction in amyotrophic lateral sclerosis.
Autonomic dysregulation is part of the complex degenerative process in amyotrophic lateral sclerosis (ALS). To investigate this, sweating was examined at rest in 39 patients with ALS in comparison with a control group. Sweat was collected over a 30 second period over the thenar and hypothenar eminences and on the sole of the foot, using a commercial device based on vapour pressure gradient. The...
متن کاملIs Amyotrophic Lateral Sclerosis a Mitochondrial Channelopathy?
SOD1 is a cause of the fatal, paralytic disorder ALS. Although mechanisms underlying mutant SOD1 neurotoxicity remain uncertain, this protein associates with mitochondria. In this issue of Neuron, Israelson et al. show that mutant SOD1 binds and inhibits the mitochondrial channel VDAC1. This finding sheds light onto possible molecular links between mutant SOD1, mitochondrial dysfunction, and sp...
متن کاملAmyotrophic Lateral Sclerosis in a Patient with Behçet’s Disease
Behçet’s disease is a multisystem vasculitis. Its neurological involvement mostly includes parenchymal and non-parenchymal central nervous system manifestations. Peripheral nervous system presentations are rare. A 32-yr-old male patient who fulfilled the international study group criteria for Behçet’s disease, referred to our center with walking difficulty and repeated falling downs. Neurologi...
متن کاملMutant Cu/Zn-Superoxide Dismutase Induced Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis
Mutations in Cu/Zn superoxide dismutase (SOD1) gene are linked to the motor neuron death in familial amyotrophic lateral sclerosis (FALS). More than 100 missense mutations have been described to cause the disease and are distributed throughout the whole 153 amino acid sequence of SOD1 molecule (Valentine et al., 2005; Boillée et al., 2006). Mutant SOD1 molecules can be grouped according to thei...
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: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
سال: 2010
ISSN: 0925-4439
DOI: 10.1016/j.bbadis.2009.08.012