Neurobiology of Disease Abnormal Intracellular Calcium Signaling and SNARE- Dependent Exocytosis Contributes to SOD1G93A Astrocyte- Mediated Toxicity in Amyotrophic Lateral Sclerosis
نویسندگان
چکیده
Hibiki Kawamata,1 Seng Kah Ng,3 Natalia Diaz,3 Suzanne Burstein,1 Lydie Morel,3 Alexandra Osgood,3 Brittany Sider,1 Haruki Higashimori,3 Philip G. Haydon,3,4 Giovanni Manfredi,1,2 and Yongjie Yang3,4 1Brain and Mind Research Institute and 2Department of Neurology, Weill Medical College of Cornell University, New York, New York 10065, and 3Department of Neuroscience and 4Sackler School of Biomedical Sciences, Tufts University, Boston, Massachusetts 02111
منابع مشابه
Abnormal intracellular calcium signaling and SNARE-dependent exocytosis contributes to SOD1G93A astrocyte-mediated toxicity in amyotrophic lateral sclerosis.
Motor neurons are progressively and predominantly degenerated in ALS, which is not only induced by multiple intrinsic pathways but also significantly influenced by the neighboring glial cells. In particular, astrocytes derived from the SOD1 mutant mouse model of ALS or from human familial or sporadic ALS patient brain tissue directly induce motor neuron death in culture; however, the mechanisms...
متن کاملTime-Point Dependent Activation of Autophagy and the UPS in SOD1G93A Mice Skeletal Muscle
Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease characterized by a selective loss of motor neurons together with a progressive muscle weakness. Albeit the pathophysiological mechanisms of the disease remain unknown, growing evidence suggests that skeletal muscle can be a target of ALS toxicity. In particular, the two main intracellular degradation mechanisms, autophagy ...
متن کاملSelective mitochondrial Ca2+ uptake deficit in disease endstage vulnerable motoneurons of the SOD1G93A mouse model of amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis is a progressive neurodegenerative disease that targets some somatic motoneuron populations, while others, e.g. those of the oculomotor system, are spared. The pathophysiological basis of this pattern of differential vulnerability, which is preserved in a transgenic mouse model of amyotrophic lateral sclerosis (SOD1(G93A)), and the mechanism of neurodegeneration in...
متن کاملThe Cu, Zn Superoxide Dismutase: Not Only a Dismutase Enzyme
The Cu,Zn superoxide dismutase (SOD1) is an ubiquitary cytosolic dimeric carbohydrate free molecule, belonging to a family of isoenzymes involved in the scavenger of superoxide anions. This effect certainly represents the main and well known function ascribed to this enzyme. Here we highlight new aspects of SOD1 physiology that point out some inedited effects of this enzyme in addition to the c...
متن کاملKnocking down metabotropic glutamate receptor 1 improves survival and disease progression in the SOD1G93A mouse model of amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a late-onset fatal neurodegenerative disease reflecting degeneration of upper and lower motoneurons (MNs). The cause of ALS and the mechanisms of neuronal death are still largely obscure, thus impairing the establishment of efficacious therapies. Glutamate (Glu)-mediated excitotoxicity plays a major role in MN degeneration in ALS. We recently demonstrated ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره شماره
صفحات -
تاریخ انتشار 2014