Related Experiment Video
Updated: Dec 14, 2025

08:35
In Situ Immunofluorescent Staining of Autophagy in Muscle Stem Cells
Published on: June 12, 2017
10.5K
Autophagy and Motor Neuron Diseases
Xiaojie Zhang1, Kang Yang2, Weidong Le3
1Department of Neurology, Shanghai Jiaotong University Affiliated Sixth People's Hospital, Shanghai, China.
Advances in Experimental Medicine and Biology
|July 17, 2020
Summary
Motor neuron diseases (MND) involve the progressive loss of motor neurons, impacting muscle function. This chapter explores how autophagy alterations contribute to the neurodegenerative mechanisms in amyotrophic lateral sclerosis (ALS).
Area of Science:
- Neuroscience
- Cell Biology
- Genetics
Background:
- Motor neuron diseases (MND) are fatal neurodegenerative conditions affecting motor system pathways.
- Motor neurons are classified as upper or lower, with distinct consequences upon degeneration.
- Key MND types include amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), progressive muscular atrophy (PMA), and primary lateral sclerosis (PLS).
Purpose of the Study:
- To summarize the alterations in autophagy observed in motor neurons.
- To elucidate the contribution of autophagy dysfunction to the pathogenesis of ALS.
Main Methods:
- Literature review focusing on autophagy studies in MND, particularly ALS.
- Analysis of research on the role of autophagy in motor neuron degeneration.
Main Results:
- Autophagy, a cellular degradation process, is frequently dysregulated in ALS.
- Altered autophagy impacts motor neuron survival and function, contributing to disease progression.
Conclusions:
- Understanding autophagy alterations is crucial for deciphering ALS pathogenesis.
- Targeting autophagy pathways may offer potential therapeutic strategies for ALS.
Related Concept Videos
Autophagy
5.5K
Autophagy is a self-digesting process by which a cell protects itself from threats both within and outside the cell, ranging from abnormal proteins to invading bacteria. In this process, obsolete components of the cell and invading microbes are degraded by hydrolytic enzymes active in an acidic environment of the lysosomal lumen.
An autophagic pathway consists of a series of signaling events activated in response to diverse stress and physiological conditions such as food deprivation,...
An autophagic pathway consists of a series of signaling events activated in response to diverse stress and physiological conditions such as food deprivation,...
5.5K
Delivery Pathways to the Lysosome
8.6K
Eukaryotic cells use different mechanisms to eliminate toxic waste obsolete and worn-out substances. Lysosomes play a pivotal role in this, and hence, these substances are carried to the lysosome from other parts of the cell and extracellular space through different pathways. The most elaborately studied pathways to the lysosome are the endocytic pathways.
Endocytosis
In endocytosis, the cell membrane takes up macromolecules and particles from the surrounding medium. Clathrin-mediated...
Endocytosis
In endocytosis, the cell membrane takes up macromolecules and particles from the surrounding medium. Clathrin-mediated...
8.6K
Lysosomal Hydrolases
4.3K
Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
4.3K
Autophagic Cell Death
4.1K
Christian de Duve discovered “autophagy,” a process in which cellular components are engulfed by membrane-bound organelles called autophagosomes. The autophagosomes then fuse with lysosomes to digest the enclosed contents. Autophagy is generally activated in cells to prevent cell death. However, cell death is triggered when the damage is beyond repair.
Autophagy and Apoptosis
Autophagy can activate apoptosis. In normal conditions, the autophagy activating protein Beclin-1 and...
Autophagy and Apoptosis
Autophagy can activate apoptosis. In normal conditions, the autophagy activating protein Beclin-1 and...
4.1K
Cross-bridge Cycle
121.5K
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.
121.5K
Parkinson's Disease: Overview
1.5K
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...
1.5K

