F082 Lipid metabolism plays a fundamental role in the progression of Huntington disease

<h3></h3> Patients with Huntington disease (HD) exhibit motor symptoms, cognitive impairment, psychological deterioration and behavioral changes. Furthermore, they also experience a strong loss of weight. The results previously obtained in our laboratory revealed that some molecules involved in the synthesis and degradation of fats have an impact on proteostasis by altering PolyQ aggregation patterns. Some lipids and their metabolism are key factors to a wide range of cellular processes involved in health maintenance, such as control of neuroinflammation and immunosuppressive response. Based on the evidence, we believe that alterations in lipid metabolism play a fundamental role in the progression of HD, inducing an alteration in proteostasis. We are motivated to demonstrate that alterations in lipids can be detected prior to the appearance of symptoms. The potential regulation of these compounds or synthesis pathways could modulate the progression of the disease. To get this, two experimental groups were formed, controls and HD patients, following exclusion criteria specifically related to fat metabolism, divided equally by gender and age. Lipidomic was performed on serum by liquid chromatography. After the analysis, significant differences were identified in at least 247 lipids between the two groups studied. The main components distinguishing the two groups were primarily compounds from the triglyceride and glycerophosphocholine families which have previously been correlated with other pathologies such as Parkinson’s disease or Alzheimer’s. The identification of these lipids could help in the development of corrective supplementing therapies as well as the discovery of genes that act as critical regulators.

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Published
2024-09-01
DOI
https://doi.org/10.1136/jnnp-2024-ehdn.200
Primary Topic
Genetic Neurodegenerative Diseases
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article
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article

F082 Lipid metabolism plays a fundamental role in the progression of Huntington disease

Julia Tortajada-Pérez, Andrea del Valle Carranza, Amadeo Ten‐Esteve, Rafael P. Vázquez‐Manrique et al.
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Genetic Neurodegenerative Diseases
article

F082 Lipid metabolism plays a fundamental role in the progression of Huntington disease

Julia Tortajada-Pérez, Andrea del Valle Carranza, Amadeo Ten‐Esteve, Rafael P. Vázquez‐Manrique, Carmen Peiró Velert, Marta Roca, Agustín Lahoz-Rodriguez, Francisco Castera-Brugada
article en

Abstract

<h3></h3> Patients with Huntington disease (HD) exhibit motor symptoms, cognitive impairment, psychological deterioration and behavioral changes. Furthermore, they also experience a strong loss of weight. The results previously obtained in our laboratory revealed that some molecules involved in the synthesis and degradation of fats have an impact on proteostasis by altering PolyQ aggregation patterns. Some lipids and their metabolism are key factors to a wide range of cellular processes involved in health maintenance, such as control of neuroinflammation and immunosuppressive response. Based on the evidence, we believe that alterations in lipid metabolism play a fundamental role in the progression of HD, inducing an alteration in proteostasis. We are motivated to demonstrate that alterations in lipids can be detected prior to the appearance of symptoms. The potential regulation of these compounds or synthesis pathways could modulate the progression of the disease. To get this, two experimental groups were formed, controls and HD patients, following exclusion criteria specifically related to fat metabolism, divided equally by gender and age. Lipidomic was performed on serum by liquid chromatography. After the analysis, significant differences were identified in at least 247 lipids between the two groups studied. The main components distinguishing the two groups were primarily compounds from the triglyceride and glycerophosphocholine families which have previously been correlated with other pathologies such as Parkinson’s disease or Alzheimer’s. The identification of these lipids could help in the development of corrective supplementing therapies as well as the discovery of genes that act as critical regulators.

Hospital Universitari i Politècnic La Fe (ES), Centre for Biomedical Network Research on Rare Diseases (ES), Centro de Investigación Biomédica en Red (ES), Instituto de Investigación Sanitaria La Fe (ES)
Openalex Percentile: Top 84%
Genetic Neurodegenerative Diseases
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