Integrative hypothesis for Huntington´s disease: a brief review of experimental evidence

Title:

Integrative hypothesis for Huntington´s disease: a brief review of experimental evidence

Creator:

Pérez-de la Cruze, V. and Santamaría, A.

Identifier:

https://cdk.lib.cas.cz/client/handle/uuid:b183f957-e652-4527-b64d-c92ce410a212
uuid:b183f957-e652-4527-b64d-c92ce410a212
issn:0862-8408

Subject:

Patologie. Klinická medicína, biochemie, Huntingtonova nemoc, energetický metabolismus, oxidační stres, záněty, biochemistry, Huntington’s disease, energy metabolism, oxidative stress, inflammations, excitotoxicita, huntingtin aggregation, excitotoxicity, 14, and 616

Type:

article, články, model:article, and TEXT

Format:

print, bez média, and svazek

Description:

Huntington’s disease (HD) is a demential, neurodegenerative inheritable disease affecting middle-aged patients. HD is characterized by uncontrolled choreiform movements, psychiatric symptoms and cognitive decline. Histopathological changes in HD brains reveal a considerable damage to basal ganglia, particularly affecting middle-sized spiny neurons from the caudate-putamen region. Neurochemical changes are specifically oriented to deplete GABAergic and cholinergic systems, while molecular alterations include an increased expression of CAG trinucleotide at exon 1 from the huntingtin (htt) gene, as well as aggregation of mutant htt. Although several hypotheses regarding the mechanisms by which neurotoxicity is triggered in HD brains have been suggested on the basis of experimental evidence, so far it remains not clear which of them are predominant or whether they are complementary. Recent experimental evidence through transgenic mice models reveal an interesting inter action between expanded CAG triplets, mutant htt, and the increase in toxic metabolites from the kynurenine pathway. Further evidence supports the assumption that different toxic mechanisms (i.e. excitotoxicity, energy metabolism impairment, inflammatory events, oxidative stress, etc.) are confluent and depend on each other. In this review we will briefly summarize some of those findings and propose a final integrative hypothesis for HD., V. Pérez-de la Cruz, A. Santamaría., and Obsahuje bibliografii a bibliografické odkazy

Language:

English

Rights:

http://creativecommons.org/licenses/by-nc-sa/4.0/
policy:public

Source:

Physiological research | 2007 Volume:56 | Number:5

Harvested from:

CDK

Metadata only:

false