Journal Article Review Epublish 11 citations
PubMed: 35682773 DOI: 10.3390/ijms23116089
A New Perspective on Huntington's Disease: How a Neurological Disorder Influences the Peripheral Tissues.
Int J Mol Sci 23(11)
Authors: Laura Gómez-Jaramillo, Fátima Cano-Cano, María Del Carmen González-Montelongo, Antonio Campos-Caro, Manuel Aguilar-Diosdado, Ana I Arroba
Affiliations
- Undad de Investigación, Instituto de Investigación e Innovación en Ciencias Biomédicas de la Provincia de Cádiz (INiBICA), 11002 Cádiz, Spain.
- Área de Genética, Departamento de Biomedicina, Biotecnología y Salud Pública, Universidad de Cádiz, 11002 Cádiz, Spain.
- Departamento de Endocrinología y Nutrición, Hospital Universitario Puerta del Mar, Universidad de Cádiz, 11002 Cádiz, Spain.
Abstract
Huntington's disease (HD) is a neurodegenerative disorder caused by a toxic, aggregation-prone expansion of CAG repeats in the HTT gene with an age-dependent progression that leads to behavioral, cognitive and motor symptoms. Principally affecting the frontal cortex and the striatum, mHTT disrupts many cellular functions. In fact, increasing evidence shows that peripheral tissues are affected by neurodegenerative diseases. It establishes an active crosstalk between peripheral tissues and the brain in different neurodegenerative diseases. This review focuses on the current knowledge of peripheral tissue effects in HD animal and cell experimental models and identifies biomarkers and mechanisms involved or affected in the progression of the disease as new therapeutic or early diagnostic options. The particular changes in serum/plasma, blood cells such as lymphocytes, immune blood cells, the pancreas, the heart, the retina, the liver, the kidney and pericytes as a part of the blood-brain barrier are described. It is important to note that several changes in different mouse models of HD present differences between them and between the different ages analyzed. The understanding of the impact of peripheral organ inflammation in HD may open new avenues for the development of novel therapeutic targets.
MeSH
Huntington Disease, Nervous System Diseases, Blood Cells, Therapeutics, Genes, Blood-Brain Barrier, Neurodegenerative Diseases, Tissues
Cited by
- Investigating the Interplay between Cardiovascular and Neurodegenerative Disease. Biology (Basel), 2024
- Systemic Symptoms in Huntington's Disease: A Comprehensive Review. Mov Disord Clin Pract, 2024
- Molecular Research on Huntington's Disease. Int J Mol Sci, 2023
- The dual face of microglia (M1/M2) as a potential target in the protective effect of nutraceuticals against neurodegenerative diseases. Front Aging, 2023
- The updated development of blood-based biomarkers for Huntington's disease. J Neurol, 2023
- Retinal dysfunction in Huntington's disease mouse models concurs with local gliosis and microglia activation. Sci Rep, 2024