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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,...
Other Glycolytic Pathways01:24

Other Glycolytic Pathways

The pentose phosphate pathway (PPP) operates in parallel with glycolysis, facilitating the metabolism of both pentoses and glucose. This pathway consists of two distinct phases: the oxidative and non-oxidative phases. While it does not directly generate ATP, the intermediates formed during the process can integrate into glycolysis, contributing to cellular energy metabolism when required.Oxidative Phase: NADPH ProductionThe oxidative phase of the pentose phosphate pathway is primarily...

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Related Experiment Video

Updated: May 28, 2026

Measuring Glucose Uptake in Drosophila Models of TDP-43 Proteinopathy
07:07

Measuring Glucose Uptake in Drosophila Models of TDP-43 Proteinopathy

Published on: August 3, 2021

ROS-HIF1α-driven glycolytic reprogramming sustains ATP production in Huntington's disease.

Ching-Wen Wu1, Ching-Pang Chang1, Dennis W Hwang2

  • 1Biomedical Translation Research Center, Academia Sinica, Taipei, Taiwan; Institute of Biomedical Sciences, Academia Sinica, Taipei, Taiwan.

Neurobiology of Disease
|May 26, 2026
PubMed
Summary
This summary is machine-generated.

Huntington's disease (HD) involves elevated ATP levels due to a metabolic switch. Oxidative stress activates hypoxia-inducible factor-1α (HIF1α), boosting glycolysis to compensate for mitochondrial dysfunction.

Keywords:
Energy metabolismGlucose uptakeGlycolysisHIF1αHuntington's diseaseMetabolic resilienceOxidative stress

Related Experiment Videos

Last Updated: May 28, 2026

Measuring Glucose Uptake in Drosophila Models of TDP-43 Proteinopathy
07:07

Measuring Glucose Uptake in Drosophila Models of TDP-43 Proteinopathy

Published on: August 3, 2021

Area of Science:

  • Neuroscience
  • Metabolic research
  • Genetics

Background:

  • Huntington's disease (HD) is a progressive neurodegenerative disorder.
  • Mitochondrial dysfunction and impaired energy metabolism are implicated in HD pathogenesis.
  • Previous assumptions suggested diminished ATP levels in HD.

Purpose of the Study:

  • To investigate the bioenergetic profile in the striatum of Huntington's disease models.
  • To elucidate the mechanisms behind altered energy metabolism in HD.
  • To identify potential therapeutic targets related to metabolic adaptation.

Main Methods:

  • Integrative metabolomics and gene expression profiling in R6/2 HD mice.
  • Pharmacological inhibition of glycolysis and hypoxia-inducible factor-1α (HIF1α).
  • In vivo dynamic glucose-enhanced (DGE) MRI and single-nucleus RNA sequencing.

Main Results:

  • ATP levels were elevated, not diminished, in the striatum of R6/2 HD mice.
  • Increased reactive oxygen species stabilized HIF1α, enhancing glucose uptake and glycolysis.
  • Inhibition of glycolysis or HIF1α abolished ATP elevation, confirming HIF1α-dependent compensation.
  • Coordinated metabolic reprogramming was observed across neuronal and glial cells.

Conclusions:

  • HD involves an oxidative stress-triggered metabolic switch sustaining ATP production.
  • HIF1α-dependent glycolysis compensates for mitochondrial deficits in HD.
  • This metabolic adaptation redefines understanding of bioenergetic changes in neurodegenerative diseases.