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

166
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...
166
The Nucleolus02:55

The Nucleolus

8.6K
The nucleolus is the most prominent substructure of the nucleus. When it was first discovered, it was considered to be an isolated organelle that forms fibrils and granules. In 1931, the relationship between the nucleolus and chromosomes was first described by Heitz. He observed that the appearance and size of nucleolus varies depending on the stage of the cell cycle. He also noticed constricted regions on different chromosomes clustered together at definite cell cycle stages. These regions,...
8.6K
Lysosomal Hydrolases01:22

Lysosomal Hydrolases

3.6K
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,...
3.6K
Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

34
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...
34
Nucleosome Remodeling02:54

Nucleosome Remodeling

8.8K
Nucleosomes are the basic units of chromatin compaction. Each nucleosome consists of the DNA bound tightly around a histone core, which makes the DNA inaccessible to DNA binding proteins such as DNA polymerase and RNA polymerase. Hence, the fundamental problem is to ensure access to DNA when appropriate, despite the compact and protective chromatin structure.
Nucleosome remodeling complex
Eukaryotic cells have specialized enzymes called ATP-dependent nucleosome remodeling enzymes. These enzymes...
8.8K
Neural Regulation01:37

Neural Regulation

34.8K
Digestion begins with a cephalic phase that prepares the digestive system to receive food. When our brain processes visual or olfactory information about food, it triggers impulses in the cranial nerves innervating the salivary glands and stomach to prepare for food.
34.8K

You might also read

Related Articles

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

Sort by
Same author

Basal forebrain parvalbumin neuron dysfunction links network oscillation deficits to hippocampal pathology in Alzheimer's disease.

Research square·2026
Same author

Kilovoltage Energy Significantly Enhances the Therapeutic Efficacy of Low-Dose Radiation in a 3xTg-AD Mouse Model of Alzheimer's Disease.

International journal of molecular sciences·2026
Same author

Correction: Kim et al. Identification of <i>GREM-1</i> and <i>GAS6</i> as Specific Biomarkers for Cancer-Associated Fibroblasts Derived from Patients with Non-Small-Cell Lung Cancer. <i>Cancers</i> 2025, <i>17</i>, 2858.

Cancers·2026
Same author

Optimizing lymph node dissection for accurate nodal staging in early-stage esophageal squamous cell carcinoma: a multicenter study.

Esophagus : official journal of the Japan Esophageal Society·2026
Same author

Relationships between cognition and daily functioning in adults with bipolar disorder: A systematic review and multilevel meta-analysis.

Psychological bulletin·2026
Same author

Longitudinal pulmonary function changes and pulmonary complications after robot-assisted thoracoscopic versus open transthoracic esophagectomy.

The Journal of thoracic and cardiovascular surgery·2026
Same journal

Cumulative Contents.

Biochimica et biophysica acta·2020
Same journal

Molecular Basis of Disease Cumulative Contents.

Biochimica et biophysica acta·2020
Same journal

General Subjects Cumulative Contents.

Biochimica et biophysica acta·2020
Same journal

Erratum to 'on the role of exchangeable hydrogen bonds for the kinetics of P680<sup>+·</sup> Q<sub>A</sub> <sup>-·</sup> formation and P680<sup>+·</sup> Pheo<sup>-·</sup> recombination in photosystem II' [Biochim. Biophys. Acta 1276 (1996) 35-44].

Biochimica et biophysica acta·2019
Same journal

Oligomeric state of the light-harvesting complexes B800-850 and B875 from purple bacterium Rubrivivax gelatinosus in detergent solution.

Biochimica et biophysica acta·2019
Same journal

Regulation of pigment content and enzyme activity in the cyanobacterium Nostoc sp. Mac grown in continuous light, a light-dark photoperiod, or darkness.

Biochimica et biophysica acta·2019
See all related articles

Related Experiment Video

Updated: May 6, 2026

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

9.0K

Nucleolar dysfunction in Huntington's disease.

Junghee Lee1, Yu Jin Hwang2, Hyun Ryu3

  • 1VA Boston Healthcare System, Boston, MA 02130, USA; Boston University, Alzheimer's Disease Center, Department of Neurology, Boston University School of Medicine, Boston, MA 02118, USA.

Biochimica Et Biophysica Acta
|November 5, 2013
PubMed
Summary
This summary is machine-generated.

Huntington's disease involves genetic mutations affecting neuronal function. This review explores how epigenetic modifications in the nucleolus, specifically involving upstream binding factor (UBF), may offer new therapeutic targets for this neurodegenerative disorder.

Keywords:
Acetylation and methylationEpigeneticsHuntington's diseaseNucleolusUpstream binding factor (UBF)rDNA transcription

More Related Videos

Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System
10:52

Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System

Published on: December 10, 2021

4.6K
Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells
08:53

Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells

Published on: May 16, 2017

8.4K

Related Experiment Videos

Last Updated: May 6, 2026

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

9.0K
Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System
10:52

Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System

Published on: December 10, 2021

4.6K
Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells
08:53

Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells

Published on: May 16, 2017

8.4K

Area of Science:

  • Neuroscience
  • Genetics
  • Epigenetics

Background:

  • Huntington's disease (HD) is a fatal neurodegenerative disorder caused by CAG repeat expansion in the huntingtin gene.
  • The precise mechanisms leading to selective medium spiny neuron loss in HD remain unclear.
  • Emerging evidence links nucleolar stress and dysfunction to HD pathogenesis, including deregulation of ribosomal DNA (rDNA) transcription.

Purpose of the Study:

  • To review the role of nucleolar stress and epigenetic modifications in Huntington's disease.
  • To discuss the impact of post-translational modifications on upstream binding factor (UBF) and rDNA transcription in HD.

Main Methods:

  • Literature review focusing on nucleolar function, epigenetic regulation, and Huntington's disease.
  • Analysis of the interplay between histone acetyltransferase, histone methyltransferase, and UBF in rDNA transcription.
  • Examination of the link between nucleolar alterations and neuronal damage in HD.

Main Results:

  • Mutant huntingtin may induce nucleolar stress, affecting rDNA transcription.
  • Epigenetic modifications, particularly those involving UBF, are implicated in the nucleolar dysfunction observed in HD.
  • Histone acetyltransferase and histone methyltransferase activities influence UBF post-translational modifications and rDNA transcription.

Conclusions:

  • Understanding the epigenetic modulation of UBF-dependent rDNA transcription in the nucleolus is crucial for HD research.
  • This knowledge may lead to the identification of novel pathological markers for HD.
  • Targeting nucleolar epigenetic pathways presents a potential therapeutic strategy for Huntington's disease.