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Mitochondria

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,...

Mitochondria

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 Membranes

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,...

Mitochondrial Membranes

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,...

Electron Transport Chain: Complex I and II

Electron Transport Chain: Complex I and II

The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
ROS generation is regulated and maintained at moderate levels necessary...

Translocation of Proteins into the Mitochondria

Translocation of Proteins into the Mitochondria

Mitochondrial precursors are translocated to the internal subcompartments via independent mechanisms involving distinct protein machineries called translocases.
Sorting of outer membrane proteins:
Mitochondrial outer membrane proteins are of two types: the transmembrane, beta-barrel porins, and the membrane-anchored, alpha-helical proteins. Beta-barrel porin precursors are translocated by the TOM complex and inserted into the outer mitochondrial membrane by the SAM complex. In contrast,...

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Related Experiment Video

Updated: Jul 9, 2026

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

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

Published on: June 30, 2023

Mitochondria and neurodegeneration.

¹Weill Medical College of Cornell University, Department of Neurology and Neuroscience, 525 East 68th Street, New York, NY 10021, USA.

Novartis Foundation Symposium

|December 14, 2007

Summary

Mitochondrial dysfunction is increasingly linked to neurodegenerative diseases like Alzheimer's, Huntington's, Parkinson's, and ALS. Targeting mitochondria offers promising therapeutic strategies for these conditions.

Area of Science:

Neuroscience
Cell Biology
Genetics

Background:

Mitochondrial dysfunction is a common factor in neurodegenerative diseases.
Aging, a major risk factor, involves mitochondrial DNA mutations and oxidative stress.
Mitochondria regulate cell death, a hallmark of neurodegeneration.

Purpose of the Study:

To explore the link between mitochondrial dysfunction and specific neurodegenerative diseases.
To highlight the role of mitochondria in disease pathogenesis.
To identify potential therapeutic targets within mitochondria.

Main Methods:

Review of existing evidence linking mitochondrial dysfunction to neurodegenerative diseases.
Examination of specific protein interactions with mitochondria in Alzheimer's, Huntington's, Parkinson's, and ALS.

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Last Updated: Jul 9, 2026

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

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Published on: June 30, 2023

Histological Examination of Mitochondrial Morphology in a Parkinson's Disease Model

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Published on: June 23, 2023

Analysis of Brain Mitochondria Using Serial Block-Face Scanning Electron Microscopy

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Published on: July 9, 2016

Analysis of the role of oxidative stress and mitochondrial biogenesis.

Main Results:

Beta-amyloid and amyloid precursor protein interact with mitochondria in Alzheimer's disease, increasing free radicals.
Down-regulation of PGC1alpha, a mitochondrial biogenesis regulator, is observed in Huntington's disease.
Genes linked to Parkinson's disease (parkin, DJ1, PINK1) are associated with oxidative stress or mitochondrial dysfunction.
Mutant superoxide dismutase interacts with mitochondria in amyotrophic lateral sclerosis.

Conclusions:

Numerous disease-specific proteins interact with mitochondria across various neurodegenerative disorders.
Therapeutic strategies targeting mitochondrial energy metabolism, free radical generation, and protein interactions show significant promise.