Related Experiment Video

Updated: May 9, 2026

Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models
08:48

Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models

Published on: June 30, 2023

Mitochondrial dysfunction and neurodegeneration in multiple sclerosis

Kimmy Su1, Dennis Bourdette, Michael Forte

  • 1Vollum Institute, Oregon Health and Science University Portland, OR, USA ; Department of Neurology, Oregon Health and Science University Portland, OR, USA.

Frontiers in Physiology
|July 31, 2013
PubMed

Insights

Mitochondrial dysfunction, driven by reactive oxygen species and calcium imbalance, contributes to neurodegeneration in multiple sclerosis (MS). Targeting the enzyme p66ShcA shows promise for mitigating this process in MS patients.

Area of Science:

  • Neuroscience
  • Immunology
  • Cell Biology

Background:

  • Multiple sclerosis (MS) is traditionally viewed as an autoimmune inflammatory disease.
  • Current treatments manage relapses but fail to halt progressive functional decline.
  • Emerging evidence suggests neurodegeneration plays a significant role in MS pathogenesis.

Purpose of the Study:

  • To review evidence supporting mitochondrial dysfunction as a key mechanism in MS neurodegeneration.
  • To investigate the role of pathological permeability transition pore (PTP) opening in MS.
  • To highlight the involvement of reactive oxygen species (ROS) and calcium dysregulation.

Main Methods:

  • Focused review of existing literature.
  • In vitro studies on mitochondrial function.
  • In vivo investigations utilizing p66ShcA as a target.

Main Results:

  • Pathological PTP opening, mediated by ROS and calcium, is implicated in mitochondrial dysfunction.
  • Mitochondrial dysfunction is a central factor in MS-related neurodegeneration.
  • The mitochondria-targeted enzyme p66ShcA plays a crucial role in this pathway.

Conclusions:

  • Mitochondrial dysfunction is a critical component of neurodegeneration in multiple sclerosis.
  • Targeting p66ShcA offers a potential therapeutic strategy for MS.
  • Understanding the interplay between PTP opening, ROS, and calcium is vital for MS research.
Keywords:
mitochondriamultiple sclerosisneurodegenerationneuronal viabilityp66ShcA

More Related Videos

Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry08:19

Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry

Published on: May 5, 2022

Comprehensive Autopsy Program for Individuals with Multiple Sclerosis09:41

Comprehensive Autopsy Program for Individuals with Multiple Sclerosis

Published on: July 19, 2019

Related Concept Videos

Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
Parkinson's Disease: Overview01:15

Parkinson's Disease: Overview

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 to...
Alzheimer Disease ll: Pathophysiology01:23

Alzheimer Disease ll: Pathophysiology

Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and microglia. Abnormal...
Mitochondria01:37

Mitochondria

Mitochondria are eukaryotic cellular organelles that are known to produce energy through a process called oxidative phosphorylation. Besides their primary function, mitochondria are involved in various cellular processes, including cell growth, differentiation, signaling, metabolism, and senescence. Age-related changes cause a decline in mitochondrial quality and integrity due to increased mitochondrial mutations and oxidative damage. Thus, aging can severely impact mitochondrial functions,...
Mitochondrial Membranes01:45

Mitochondrial Membranes

A single mitochondrion is a bean-shaped organelle enclosed by a double-membrane system. The outer membrane of mitochondria is smooth and contains many porins - the integral membrane transporters. Porins enable free diffusion of ions and small uncharged molecules through the outer mitochondrial membrane but limit the transport of molecules larger than 5000 Daltons. Further, the outer mitochondrial membrane forms a unique structure called membrane contact sites with other subcellular organelles,...