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Related Concept Videos

Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
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...
Translocation of Proteins into the Mitochondria01:19

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,...
Lysosomal Hydrolases01:22

Lysosomal Hydrolases

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,...
Electron Transport Chain: Complex I and II01:46

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...
ATP Synthase: Mechanism01:48

ATP Synthase: Mechanism

In animals, the mitochondrial F1F0 ATP synthase is the key protein that synthesizes ATP molecules through a complex catalytic mechanism. While the nuclear genome encodes the majority of ATP synthase subunits, the mitochondrial genome encodes some of the enzyme's most critical components. The formation of this multi-subunit enzyme is a complex multi-step process regulated at the level of transcription, translation, and assembly. Defects in one or more of these steps can result in decreased ATP...

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Using Live Cell STED Imaging to Visualize Mitochondrial Inner Membrane Ultrastructure in Neuronal Cell Models
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Published on: June 30, 2023

Mitochondrial dysfunction and Huntington disease.

Wei-Yan Zhang1, Zhen-Lun Gu, Zhong-Qin Liang

  • 1SooChow University School of Life Sciences; Department of Pharmacology, Soochow University School of Medicine, Suzhou 215123, China;

Neuroscience Bulletin
|August 10, 2007
PubMed
Summary

Huntington disease (HD) involves mitochondrial dysfunction, a key factor in its progression. Understanding these mechanisms is crucial for developing effective treatments for this neurodegenerative disorder.

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Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Huntington disease (HD) is an autosomal-dominant neurodegenerative disorder.
  • The genetic basis of HD involves mutations in the Huntingtin gene.
  • The precise pathogenic mechanisms underlying HD remain incompletely understood.

Purpose of the Study:

  • To review the role of mitochondrial dysfunction in the pathogenesis of Huntington disease.
  • To consolidate current knowledge on how cellular energy production defects contribute to HD.
  • To highlight potential therapeutic targets related to mitochondrial health in HD.

Main Methods:

  • Literature review of studies investigating mitochondrial function in HD models and patients.
  • Analysis of research on the Huntingtin protein's interaction with mitochondria.
  • Synthesis of data on oxidative stress, ATP production, and mitochondrial dynamics in HD.

Main Results:

  • Mitochondrial dysfunction is consistently observed across various HD models.
  • Impaired mitochondrial respiration and increased oxidative stress are key features.
  • Aberrant interactions between mutant Huntingtin and mitochondrial proteins disrupt cellular homeostasis.

Conclusions:

  • Mitochondrial dysfunction is a central player in Huntington disease pathogenesis.
  • Targeting mitochondrial pathways offers a promising therapeutic strategy for HD.
  • Further research is needed to fully elucidate the complex interplay between mutant Huntingtin and mitochondrial biology.