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,...
Alzheimer's Disease: Treatment01:22

Alzheimer's Disease: Treatment

Alzheimer's Disease (AD), a neurodegenerative disorder, is pathologically identified by amyloid plaques and neurofibrillary tangles composed of tau protein. AD pharmacotherapy aims to manage cognitive symptoms, delay disease progression, and treat behavioral symptoms. The treatment is primarily symptomatic and palliative, with no definitive disease-modifying therapy available. Cholinesterase inhibitors, including donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne), are...
Parkinson's Disease: Treatment01:24

Parkinson's Disease: Treatment

Neurodegenerative disorders, such as Parkinson's Disease (PD), involve the gradual and irreversible destruction of neurons in particular brain areas. These disorders exhibit standard features like proteinopathies, selective vulnerability of some neurons, and an interaction of intrinsic properties, genetics, and environmental influences in neural injury.
Parkinson's Disease is primarily a result of the loss of dopaminergic neurons in the substantia nigra pars compacta. The cornerstone of its...
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...
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 Disease l: Introduction01:24

Parkinson Disease l: Introduction

Parkinson’s disease is a chronic, progressive neurodegenerative disorder that primarily affects movement. It is characterized by motor symptoms such as resting tremors, muscle rigidity, bradykinesia (slowness of movement), and postural instability. Patients may notice hand tremors at rest, stiffness during movement, or a shuffling gait. In addition to motor features, non-motor symptoms include sleep disturbances, mood and behavioral changes, constipation, and cognitive impairment, all of which...

You might also read

Related Articles

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

Sort by
Same author

Complex spatial and temporally defined myelin and axonal degeneration in Huntington disease.

NeuroImage. Clinical·2018
Same author

Tract-based analysis of white matter degeneration in Alzheimer's disease.

Neuroscience·2015
Same author

CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion.

Neurology·2012
Same author

Tapping linked to function and structure in premanifest and symptomatic Huntington disease.

Neurology·2010
Same author

Hippocampal degeneration is associated with temporal and limbic gray matter/white matter tissue contrast in Alzheimer's disease.

NeuroImage·2010
Same author

Age-associated alterations in cortical gray and white matter signal intensity and gray to white matter contrast.

NeuroImage·2009

Related Experiment Video

Updated: Jun 19, 2026

Ole Isacson: Development of New Therapies for Parkinson's Disease
23:53

Ole Isacson: Development of New Therapies for Parkinson's Disease

Published on: April 29, 2007

Neuroprotective therapy for Huntington's disease: new prospects and challenges.

S M Hersch1, H D Rosas

  • 1Center for Ageing Genetics and Neurodegeneration, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, MGH East, Bldg. 114, Suite 2000, 114 16th Street, Charlestown, MA 02129-4404, USA. Hersch@helix.mgh.harvard.edu

Expert Review of Neurotherapeutics
|October 9, 2009
PubMed
Summary

Researchers have identified multiple molecular pathways leading to neuronal death in Huntington's disease (HD). Therapeutic strategies targeting these pathways are under investigation, but challenges remain in predicting efficacy and conducting trials in presymptomatic individuals.

More Related Videos

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
07:08

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

Published on: February 27, 2018

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
15:48

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

Published on: July 29, 2007

Related Experiment Videos

Last Updated: Jun 19, 2026

Ole Isacson: Development of New Therapies for Parkinson's Disease
23:53

Ole Isacson: Development of New Therapies for Parkinson's Disease

Published on: April 29, 2007

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
07:08

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

Published on: February 27, 2018

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
15:48

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

Published on: July 29, 2007

Area of Science:

  • Neuroscience
  • Genetics
  • Molecular Biology

Background:

  • Significant progress has been made in understanding the molecular underpinnings of neurodegenerative diseases since the discovery of the Huntington's disease (HD) genetic mutation.
  • Numerous pathways contributing to neuronal cell death in HD have been elucidated.

Purpose of the Study:

  • To review the identified molecular pathways involved in Huntington's disease pathogenesis.
  • To highlight the therapeutic opportunities arising from these discoveries.
  • To discuss the challenges in translating preclinical findings to human clinical trials.

Main Methods:

  • Review of scientific literature on Huntington's disease molecular pathology.
  • Analysis of therapeutic strategies targeting identified pathways.
  • Consideration of preclinical models (genetic, transgenic mouse) and human clinical trials.

Main Results:

  • Key pathways implicated in neuronal demise in HD include glutamatergic stress, metabolic and oxidative stress, apoptosis, inflammation, proteolysis, protein aggregation, and transcriptional/protein folding dysregulation.
  • Each identified pathway presents distinct therapeutic targets.
  • Various therapeutic interventions are currently being evaluated through high-throughput screening, genetic models, and human clinical trials.

Conclusions:

  • Multiple molecular targets offer potential therapeutic avenues for Huntington's disease.
  • Predicting the efficacy of preclinical studies in humans remains a significant challenge.
  • Optimizing neuroprotective trial design, especially for presymptomatic individuals, requires careful consideration of genetic information and potential risks.