Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

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,...
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,...
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,...
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,...
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...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Transsynaptic complex dysfunction in the hippocampus of Alzheimer's disease patients.

Frontiers in aging neuroscience·2026
Same author

Restoration of mitochondrial dynamics and synaptic function by mitophagy enhancers in a tauopathy cell model.

Mitochondrion·2026
Same author

Associations between posttraumatic stress and comorbidities of traumatic brain injury and substance use disorders with Alzheimer's disease in older veterans: A narrative review.

Ageing research reviews·2026
Same author

Behavioral consequences of serotonin deficiency in TPH2 knock-in mice: Implications for depression, anxiety, and cognitive dysfunction.

Journal of Alzheimer's disease : JAD·2026
Same author

Overexpression of miR-455-3p enhances mitophagy, synaptic and mitochondrial proteins in Alzheimer's disease.

Mitochondrion·2026
Same author

Serotonin-mediated regulation of mitophagy in Alzheimer's disease: Mechanistic insights and therapeutic potential.

Ageing research reviews·2025

Related Experiment Video

Updated: Jun 23, 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 structural and functional dynamics in Huntington's disease.

P Hemachandra Reddy1, Peizhong Mao, Maria Manczak

  • 1Neurogenetics Laboratory, Neuroscience Division, Oregon National Primate Research Center, West Campus, Oregon Health and Science University, Beaverton, OR 97006, USA. reddyh@ohsu.edu

Brain Research Reviews
|April 28, 2009
PubMed
Summary

Huntington's disease (HD) involves mutant huntingtin protein affecting mitochondria. Research highlights mitochondrial dysfunction, calcium imbalance, and DNA defects in HD progression, exploring potential therapeutics like Dimebon.

More Related Videos

Histological Examination of Mitochondrial Morphology in a Parkinson's Disease Model
06:07

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

Published on: June 23, 2023

Analyzing Mitochondrial Transport and Morphology in Human Induced Pluripotent Stem Cell-Derived Neurons in Hereditary Spastic Paraplegia
07:32

Analyzing Mitochondrial Transport and Morphology in Human Induced Pluripotent Stem Cell-Derived Neurons in Hereditary Spastic Paraplegia

Published on: February 9, 2020

Related Experiment Videos

Last Updated: Jun 23, 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

Histological Examination of Mitochondrial Morphology in a Parkinson's Disease Model
06:07

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

Published on: June 23, 2023

Analyzing Mitochondrial Transport and Morphology in Human Induced Pluripotent Stem Cell-Derived Neurons in Hereditary Spastic Paraplegia
07:32

Analyzing Mitochondrial Transport and Morphology in Human Induced Pluripotent Stem Cell-Derived Neurons in Hereditary Spastic Paraplegia

Published on: February 9, 2020

Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Huntington's disease (HD) is an inherited neurodegenerative disorder.
  • Mutant huntingtin (Htt) protein and its role in disease progression are key research areas.
  • Mitochondrial dysfunction is increasingly recognized in HD pathogenesis.

Purpose of the Study:

  • To review recent advancements in Huntington's disease research.
  • To focus on the role of mitochondria, calcium homeostasis, and DNA integrity in HD.
  • To discuss current mitochondrial therapeutic strategies for HD, including Dimebon.

Main Methods:

  • Review of recent scientific literature on Huntington's disease.
  • Analysis of studies focusing on mutant huntingtin protein interactions.
  • Examination of research on mitochondrial function and dysfunction in HD models.

Main Results:

  • Mutant Htt protein is linked to mitochondrial structural and functional deficits.
  • HD progression involves intracellular and intramitochondrial calcium dysregulation.
  • Mitochondrial DNA defects and impaired mitochondrial dynamics are implicated in HD.

Conclusions:

  • Mitochondrial abnormalities are central to Huntington's disease development and progression.
  • Targeting mitochondrial pathways offers potential therapeutic avenues for HD.
  • Further research into mitochondrial therapeutics like Dimebon is warranted for HD treatment.