ArticleMolecular & cellular proteomics : MCP2009
A large number of protein expression changes occur early in life and precede phenotype onset in a mouse model for huntington disease.
Article in Molecular & cellular proteomics : MCP, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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Who cites it
33 citing papers in PubMed, 76 citations in OpenAlex.
- Fingolimod Effects on Motor Function and BDNF-TrkB Signaling in a Huntington's Mouse Model Are Disease-Stage-Dependent.International journal of molecular sciences · 2026Article
- The Glutamate/GABA-Glutamine Cycle: Insights, Updates, and Advances.Journal of neurochemistry · 2025Review
- Glial Glutamine Homeostasis in Health and Disease.Neurochemical research · 2023Review
- Phosphoproteomic dysregulation in Huntington's disease mice is rescued by environmental enrichment.Brain communications · 2022Article
- Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington's Disease Model Mice.Brain sciences · 2021Article
- Mutant huntingtin disrupts mitochondrial proteostasis by interacting with TIM23.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- Novel proteomic changes in brain mitochondria provide insights into mitochondrial dysfunction in mouse models of Huntington's disease.Mitochondrion · 2019Article
- Early postnatal behavioral, cellular, and molecular changes in models of Huntington disease are reversible by HDAC inhibition.Proceedings of the National Academy of Sciences of the United States of America · 2018Article
- Proteomic analysis reveals distinctive protein profiles involved in CD8Cellular & molecular immunology · 2018Article
- Altered Aconitase 2 Activity in Huntington's Disease Peripheral Blood Cells and Mouse Model Striatum.International journal of molecular sciences · 2017Article
- Towards an Understanding of Energy Impairment in Huntington's Disease Brain.Journal of Huntington's disease · 2017Review
- The Role of Epigenetic Mechanisms in the Regulation of Gene Expression in the Nervous System.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Review
- Quantitative Proteomic Analysis Reveals Similarities between Huntington's Disease (HD) and Huntington's Disease-Like 2 (HDL2) Human Brains.Journal of proteome research · 2016Article
- Oxygen consumption deficit in Huntington disease mouse brain under metabolic stress.Human molecular genetics · 2016Article
- Proteomic Analysis of Dynein-Interacting Proteins in Amyotrophic Lateral Sclerosis Synaptosomes Reveals Alterations in the RNA-Binding Protein Staufen1.Molecular & cellular proteomics : MCP · 2016Article
- EFhd2, a Protein Linked to Alzheimer's Disease and Other Neurological Disorders.Frontiers in neuroscience · 2016Review
- Double NF1 inactivation affects adrenocortical function in NF1Prx1 mice and a human patient.PloS one · 2015Article
- Enhanced neuronal glucose transporter expression reveals metabolic choice in a HD Drosophila model.PloS one · 2015Article
- A common gene expression signature in Huntington's disease patient brain regions.BMC medical genomics · 2014Article
- New insight into neurodegeneration: the role of proteomics.Molecular neurobiology · 2014Review
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Authors and funding
15 authors at 6 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Huntington disease (HD) is fatal in humans within 15-20 years of symptomatic disease. Although late stage HD has been studied extensively, protein expression changes that occur at the early stages of disease and during disease progression have not been reported. In this study, we used a large two-dimensional gel/mass spectrometry-based proteomics approach to investigate HD-induced protein expression alterations and their kinetics at very early stages and during the course of disease. The murine HD model R6/2 was investigated at 2, 4, 6, 8, and 12 weeks of age, corresponding to absence of disease and early, intermediate, and late stage HD. Unexpectedly the most HD stage-specific protein changes (71-100%) as well as a drastic alteration (almost 6% of the proteome) in protein expression occurred already as early as 2 weeks of age. Early changes included mainly the up-regulation of proteins involved in glycolysis/gluconeogenesis and the down-regulation of the actin cytoskeleton. This suggests a period of highly variable protein expression that precedes the onset of HD phenotypes. Although an up-regulation of glycolysis/gluconeogenesis-related protein alterations remained dominant during HD progression, late stage alterations at 12 weeks showed an up-regulation of proteins involved in proteasomal function. The early changes in HD coincide with a peak in protein alteration during normal mouse development at 2 weeks of age that may be responsible for these massive changes. Protein and mRNA data sets showed a large overlap on the level of affected pathways but not single proteins/mRNAs. Our observations suggest that HD is characterized by a highly dynamic disease pathology not represented by linear protein concentration alterations over the course of disease.
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