Related Experiment Video

Updated: Aug 17, 2026

Delivery of Therapeutic Agents Through Intracerebroventricular (ICV) and Intravenous (IV) Injection in Mice
05:55

Delivery of Therapeutic Agents Through Intracerebroventricular (ICV) and Intravenous (IV) Injection in Mice

Published on: October 3, 2011

Role of anti-apoptotic Bcl-2 protein in spinal muscular atrophy

Y Tsujimoto1

  • 1Osaka University Medical School, Biomedical Research Center, Department of Medical Genetics, CREST of Japan Science and Technology Corp., Suita. tsujimot@gene.med.osaka-u.ac.jp

Journal of Neural Transmission. Supplementum
|December 29, 2000
PubMed

Insights

Apoptosis, programmed cell death, is crucial for health and disease. Bcl-2 family proteins regulate this process, and their dysfunction is linked to diseases like cancer and spinal muscular atrophy.

Area of Science:

  • Cell Biology
  • Molecular Biology
  • Medical Science

Background:

  • Apoptosis is a vital physiological process for cell elimination, implicated in development and disease.
  • Dysregulation of apoptosis contributes to cancer, autoimmune disorders, and degenerative diseases.
  • Caspases and the Bcl-2 protein family are key regulators of apoptosis.

Purpose of the Study:

  • To overview the regulatory mechanisms of Bcl-2 family proteins in cell death.
  • To elucidate the role of Bcl-2 family proteins in human diseases.
  • To specifically focus on the involvement of Bcl-2 in spinal muscular atrophy.

Main Methods:

  • Literature review of apoptosis regulation by Bcl-2 family proteins.
  • Analysis of the involvement of Bcl-2 proteins in various human pathologies.
  • Focused review on Bcl-2's role in spinal muscular atrophy pathogenesis.

Main Results:

  • Bcl-2 family proteins, comprising anti-apoptotic and pro-apoptotic members, finely tune cell death pathways.
  • Aberrant expression or function of Bcl-2 proteins is associated with cancer and neurodegenerative conditions.
  • Specific Bcl-2 family members play critical roles in the development and progression of spinal muscular atrophy.

Conclusions:

  • Understanding Bcl-2 family protein function is essential for developing therapies for apoptosis-related diseases.
  • Targeting Bcl-2 proteins offers potential therapeutic strategies for conditions like cancer and spinal muscular atrophy.
  • Further research into Bcl-2's role in neurodegeneration is warranted.

More Related Videos

Dissection of the Transversus Abdominis Muscle for Whole-mount Neuromuscular Junction Analysis06:12

Dissection of the Transversus Abdominis Muscle for Whole-mount Neuromuscular Junction Analysis

Published on: January 11, 2014

Systemic Delivery of MicroRNA Using Recombinant Adeno-associated Virus Serotype 9 to Treat Neuromuscular Diseases in Rodents06:51

Systemic Delivery of MicroRNA Using Recombinant Adeno-associated Virus Serotype 9 to Treat Neuromuscular Diseases in Rodents

Published on: August 10, 2018

Related Concept Videos

The Intrinsic Apoptotic Pathway01:31

The Intrinsic Apoptotic Pathway

Internal cellular stress, such as cellular injury or hypoxia, triggers intrinsic apoptosis. The B-cell lymphoma 2 (Bcl-2) family of proteins are the primary regulators of the intrinsic apoptotic pathway. For example, during DNA damage, checkpoint proteins, such as Ataxia Telangiectasia Mutated (ATM protein) and Checkpoints Factor-2 (Chk2) proteins, are activated. These proteins phosphorylate p53 which further activates pro-apoptotic proteins, such as Bax, Bak, PUMA, and Noxa, and inhibits...
Satellite Stem Cells and Muscular Dystrophy01:21

Satellite Stem Cells and Muscular Dystrophy

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...
Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Botulism01:22

Botulism

Botulism is a life-threatening neuroparalytic condition caused by botulinum neurotoxin, which is produced by the bacterium Clostridium botulinum, a Gram-positive, spore-forming, obligate anaerobe.In adults, the toxin enters the body in different ways: in foodborne botulism, the preformed toxin is absorbed in the intestine. In wound botulism, spores grow in injured tissue and release the toxin into the blood. Infant botulism differs mechanistically from adult forms. In infants, botulism commonly...
Cellular Injury V: Apoptosis and Autophagy01:22

Cellular Injury V: Apoptosis and Autophagy

Cells respond to damage and stress through highly coordinated processes that decide whether they survive or undergo controlled self-destruction. Two major pathways involved in this regulation are apoptosis, a type of programmed cell death, and autophagy, a survival mechanism that helps cells adapt to adverse conditions.ApoptosisApoptosis removes aged or injured cells to maintain tissue balance. During this process, the cell shrinks, chromatin condenses and fragments, and membrane-bound...
Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...