Reactive Species in Huntington Disease: Are They Really the Radicals You Want to Catch?

Data de publicació: 01/07/2020 Data Ahead of Print: 02/07/2020

Autors de IIS La Fe

Grups d'Investigació

Biomedicina Molecular, Celular y Genómica

Leader

José María Millán Salvador
Unidad mixta de investigación en enfermedades raras (CIPF)

Leader

José María Millán Salvador

Abstract

Huntington disease (HD) is a neurodegenerative condition and one of the so-called rare or minority diseases, due to its low prevalence (affecting 1-10 of every 100,000 people in western countries). The causative gene,HTT, encodes huntingtin, a protein with a yet unknown function. Mutant huntingtin causes a range of phenotypes, including oxidative stress and the activation of microglia and astrocytes, which leads to chronic inflammation of the brain. Although substantial efforts have been made to find a cure for HD, there is currently no medical intervention able to stop or even delay progression of the disease. Among the many targets of therapeutic intervention, oxidative stress and inflammation have been extensively studied and some clinical trials have been promoted to target them. In the present work, we review the basic research on oxidative stress in HD and the strategies used to fight it. Many of the strategies to reduce the phenotypes associated with oxidative stress have produced positive results, yet no substantial functional recovery has been observed in animal models or patients with the disease. We discuss possible explanations for this and suggest potential ways to overcome it.

Dades de la publicació

ISSN/ISSNe:: 2076-3921, 2076-3921
Tipus:: Review
Pàgines:: -
DOI:: 10.3390/antiox9070577
PubMed:: 32630706
Factor d'Impacte:: 1,067 SCImago ℠
Quartil:: Q2 SCImago ℠

Documents

No hi ha documents

Mètriques

Filiacions

Bono-Yagüe J:: Instituto de Investigación. Inst Invest Sanitaria La Fe, Lab Mol Cellular & Genom Biomed, Valencia 46026, Spain

Instituto de Investigación. Joint Unit Rare Dis IIS La Fe CIPF, Valencia 46026, Spain
Gómez-Escribano AP:: Instituto de Investigación. Inst Invest Sanitaria La Fe, Lab Mol Cellular & Genom Biomed, Valencia 46026, Spain

Instituto de Investigación. Joint Unit Rare Dis IIS La Fe CIPF, Valencia 46026, Spain

Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid 28029, Spain
Millán JM:: Instituto de Investigación. Inst Invest Sanitaria La Fe, Lab Mol Cellular & Genom Biomed, Valencia 46026, Spain

Instituto de Investigación. Joint Unit Rare Dis IIS La Fe CIPF, Valencia 46026, Spain

Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid 28029, Spain
Vázquez-Manrique RP:: Instituto de Investigación. Inst Invest Sanitaria La Fe, Lab Mol Cellular & Genom Biomed, Valencia 46026, Spain

Instituto de Investigación. Joint Unit Rare Dis IIS La Fe CIPF, Valencia 46026, Spain

Ctr Invest Biomed Red Enfermedades Raras CIBERER, Madrid 28029, Spain

Keywords

Huntington disease; huntingtin; neurodegeneration; oxidative stress; inflammation; microglia; astrocytes; free radicals; antioxidants; C; elegans; Drosophila; mouse models; clinical trials

Projectes associats

CARACTERIZACION MOLECULAR DE PACIENTES CON SINDROME DE USHER. ESTUDIO DE LOS GENES RESPONSABLES: CDH23 Y PCDH15 Y CANDIDATOS: WHRN, CXADR Y PDZK7

Investigador Principal: JOSÉ MARÍA MILLÁN SALVADOR

PI07/0558 . INSTITUTO DE SALUD CARLOS III; FUNDACIÓN PARA LA INVESTIGACIÓN DEL HOSPITAL UNIVERSITARIO LA FE DE LA COMUNIDAD VALENCIANA . 2008

MOLECULAR MAECHANISMS OF RETINAL DEGENERATION IN HUMANS WITH RETINITIS PIGMENTOSA. ROLE OF INFLAMMATION, OXIDATIVE STRESS AND GLIAL ACTIVATION

CP09/00118 (MIGUEL SERVET) . INSTITUTO DE SALUD CARLOS III; FUNDACIÓN PARA LA INVESTIGACIÓN DEL HOSPITAL UNIVERSITARIO LA FE DE LA COMUNIDAD VALENCIANA . 2010

RED DE BIOBANCOS (BIOBANCOS)

RD09/0076/00021 . INSTITUTO DE SALUD CARLOS III; FUNDACIÓN PARA LA INVESTIGACIÓN DEL HOSPITAL UNIVERSITARIO LA FE DE LA COMUNIDAD VALENCIANA . 2010

CARACTERIZACION MOLECULAR EN PACIENTES CON SINDROME DE USHER TIPO I MEDIANTE LA APLICACION DE LAS TECNICAS DE SECUENCIACION Y CGH-ARRAY EN GENES RESPONSABLES Y GENES CANDIDATOS

FPU_AP2009-3344 . MINISTERIO DE EDUCACION, CULTURA Y DEPORTE; FUNDACIÓN PARA LA INVESTIGACIÓN DEL HOSPITAL UNIVERSITARIO LA FE DE LA COMUNIDAD VALENCIANA . 2010