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 Experiment Videos

Quantitative neuropathological changes in presymptomatic Huntington's disease.

E Gómez-Tortosa1, M E MacDonald, J C Friend

  • 1Department of Neurology, Massachusetts General Hospital, Boston, USA.

Annals of Neurology
|February 24, 2001
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

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

Sort by
Same author

Plasma progranulin levels in cortical dementia phenotypes with asymmetric perisylvian atrophy.

European journal of neurology·2013
Same author

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

Neurology·2012
Same author

Herbicide exposure modifies GSTP1 haplotype association to Parkinson onset age: the GenePD Study.

Neurology·2006
Same author

BDNF genetic variants are associated with onset age of familial Parkinson disease: GenePD Study.

Neurology·2005
Same author

Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium.

Neurology·2005
Same author

Hypogonadotropic hypogonadism and cleft lip and palate caused by a balanced translocation producing haploinsufficiency for FGFR1.

Journal of medical genetics·2005
Same journal

AQP4 and MOG Characterize the Autoantibody Landscape of Checkpoint Blockade-Induced Optic Neuritis.

Annals of neurology·2026
Same journal

Five Issues of Artificial Intelligence in Science: Sailing the Ship of Theseus.

Annals of neurology·2026
Same journal

Reply to "Clinical Value of Aneurysm Wall Enhancement in Unruptured Intracranial Aneurysm".

Annals of neurology·2026
Same journal

Clinical Value of Aneurysm Wall Enhancement in Unruptured Intracranial Aneurysm.

Annals of neurology·2026
Same journal

Imaging of Neurovascular Compression in Thoracic Outlet Syndrome.

Annals of neurology·2026
Same journal

Reply to "Methodological Challenges in Interpreting SAA-Defined Imaging Subgroups in Parkinson's Disease".

Annals of neurology·2026
See all related articles

Presymptomatic individuals carrying the Huntington

Area of Science:

  • Neuroscience
  • Genetics
  • Pathology

Background:

  • Huntington's disease (HD) is a neurodegenerative disorder.
  • Pathology in HD typically first appears in the caudate nucleus.
  • Early detection of cellular changes is crucial for understanding disease progression.

Purpose of the Study:

  • To investigate morphometric changes in the caudate nucleus of presymptomatic Huntington's disease (HD) gene carriers.
  • To identify cellular differences between HD gene carriers and non-carriers before symptom onset.

Main Methods:

  • Morphometric analysis of 16 brain specimens from individuals at risk for HD.
  • Cell counting of neurons, oligodendrocytes, astrocytes, and microglia in the caudate nucleus.
  • Ubiquitin immunostaining to detect intranuclear inclusions.

Related Experiment Videos

Main Results:

  • Presymptomatic HD gene carriers showed a significantly increased density of oligodendrocytes in the caudate nucleus (40.0 cells/field) compared to non-carriers (21.3 cells/field).
  • No significant differences in neuron, astrocyte, or microglial cell density were observed between carriers and non-carriers.
  • Intranuclear inclusions were found in 3/3 gene carriers, including one with 37 CAG repeats who died 30 years before the expected clinical onset.

Conclusions:

  • An increased oligodendrocyte density in the caudate nucleus may be an early indicator of Huntington's disease, potentially reflecting a developmental effect of the HD gene.
  • These findings suggest that cellular changes in HD can occur decades before clinical symptoms manifest.
  • The observed oligodendrocyte increase is unlikely due to atrophy and may represent an early pathogenic mechanism in HD.