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関連する概念動画

Cross-bridge Cycle01:26

Cross-bridge Cycle

123.5K
As muscle contracts, the overlap between the thin and thick filaments increases, decreasing the length of the sarcomere—the contractile unit of the muscle—using energy in the form of ATP. At the molecular level, this is a cyclic, multistep process that involves binding and hydrolysis of ATP, and movement of actin by myosin.
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Parkinson's Disease: Overview01:15

Parkinson's Disease: Overview

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Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
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Satellite Stem Cells and Muscular Dystrophy01:21

Satellite Stem Cells and Muscular Dystrophy

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Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
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Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
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Chemical Synapses01:26

Chemical Synapses

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Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
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Chemical Synapses01:26

Chemical Synapses

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Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
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ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
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ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

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アミオトロフィック・ラテラル・スクレロス

Michael A van Es1, Orla Hardiman2, Adriano Chio3

  • 1Department of Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, the Netherlands.

Lancet (London, England)
|May 30, 2017
PubMed
まとめ
この要約は機械生成です。

アミオトロフィック・ラテラル・スクレロシス (ALS) はモーターニューロン喪失を伴うもので,前頭葉性認知症と特徴を共有しています. ALSの異質性を理解することは この神経変性疾患の効果的な治療法の開発の鍵です

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ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
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科学分野:

  • 神経科学
  • 遺伝学
  • 神経学

背景:

  • アミオトロフィック横筋硬化症 (ALS) は,モーターニューロンの喪失によって特徴づけられる進行性神経変性疾患である.
  • ALSは前頭葉性認知症 (FTD) と重要な病理学的特徴を共有し,多くの患者は重複する症状を示しています.
  • ALSの病因は複雑で 多様な遺伝子と病理学的経路が関与し,その異質性に対処する必要性を強調しています.

研究 の 目的:

  • ALS の現在の臨床的および診断的アプローチの包括的な概要を提供します.
  • ALSの遺伝学と病気のメカニズムを理解するための最近の科学的進歩を要約します.
  • 新しい治療戦略と ALS 管理におけるバイオマーカーの役割を調査する.

主な方法:

  • ALSとFTDの臨床試験と診断基準のレビュー
  • ALSに関連する重要な遺伝子と変異を特定する遺伝子研究の分析.
  • 細胞および動物モデルを含む様々な疾患モデルをALS研究のために検討する.
  • ALSのバイオマーカー発見と検証の研究の評価
  • ALSを標的とした臨床前および臨床的治療戦略の評価

主要な成果:

  • ALSの多様性は 遺伝的・分子的多様性によって引き起こされ 治療の開発に重大な課題をもたらします
  • 遺伝学の研究の進歩により,多くの寄与遺伝子が特定されていますが,それらの相互作用に関する統一された理解はまだ進化しています.
  • バイオマーカーの研究は 病気の進行の診断とモニタリングの改善に 期待を寄せています
  • ALSの病理学的側面を対象に複数の治療戦略が研究されています.

結論:

  • ALSの効果的な治療には 病気の根底にある多様性を考慮した 個別的なアプローチが必要です
  • ALSの治療を進めるために 遺伝学やバイオマーカーや 新しい治療目標の研究を継続することが重要です
  • ALSとFTDの重複を理解することで,共通の疾患メカニズムと治療の可能性に関する新しい洞察が得られます.