Pathogenesis of amyotrophic lateral sclerosis
نویسندگان
چکیده
منابع مشابه
Amyotrophic lateral sclerosis: pathogenesis.
Amyotrophic lateral sclerosis (ALS) is a devastating paralytic disorder caused by motor neuron degeneration. A subgroup of familial cases arises from mutations in the gene encoding cytosolic superoxide dismutase (SOD1). This review considers insight now being gained into ALS pathogenesis from the study of mutant SOD1 protein and its possible mechanisms of adverse effect on nerve cells. Also dis...
متن کاملPathogenesis of amyotrophic lateral sclerosis.
INTRODUCTION Amyotrophic lateral sclerosis (ALS) or motor neuron disease is a rapidly progressive neurodegenerative disorder. The primary involvement is of motor neurons in the brain, spinal cord and peripherally. There is secondary weakness of muscles and primary involvement of other brain regions, especially involving cognition. SOURCES OF DATA Peer-reviewed journal articles and reviews. Pu...
متن کاملPathogenesis of Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis is a devastating neurodegenerative disease affecting both upper and lower motor neuron. Despite extensive research the primary cause of the disease has not been indentified and the causative treatment is lacking. The present article describes mechanisms involved in the disease development and progression, including oxidative stress, excitotoxicity, mitochondrial dy...
متن کامل[D-serine in the pathogenesis of amyotrophic lateral sclerosis].
KIF1Bは哺乳類のキネシンスーパーファミリーの一つ である.KIF1Bとアミノ酸配列上相同性の高い KIF1サブ ファミリーには KIF1A,KIF1B,KIF1Cが属し,線虫ホモ ログは UNC104である.KIF1サブファミリーは,一般的 に二量体である他のキネシンとは異なり,微小管に結合し ていない状態では単量体として存在すると考えられてい る.この興味深い特徴のため,微小管上を移動する際の メカニズムに関して,単量体あるいは二量体として機能す るかを含めて多くの研究がなされている. KIF1Bは1150アミノ酸,130kDaのタンパク質として 報告された(図1A,KIF1Bα).その後の研究により, 1770アミノ酸,200kDaのアイソフォームの存在が発見さ れた(KIF1Bβ).両者はアミノ末端側660アミノ酸につ いて全く同一であり,選択的スプライシングによって生じ ...
متن کاملGlycosphingolipids are modulators of disease pathogenesis in amyotrophic lateral sclerosis.
Recent genetic evidence suggests that aberrant glycosphingolipid metabolism plays an important role in several neuromuscular diseases including hereditary spastic paraplegia, hereditary sensory neuropathy type 1, and non-5q spinal muscular atrophy. Here, we investigated whether altered glycosphingolipid metabolism is a modulator of disease course in amyotrophic lateral sclerosis (ALS). Levels o...
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ژورنال
عنوان ژورنال: British Medical Bulletin
سال: 2016
ISSN: 0007-1420,1471-8391
DOI: 10.1093/bmb/ldw026