Journal Article (Review Article)

DZNE-2020-02683

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Neurodegenerative processes in Huntington's disease.

Bano, D. (First author)DZNE* ; Zanetti, F.DZNE* ; Mende, Y.DZNE* ; Nicotera, P. (Last author)DZNE*

2011
Nature Publishing Group London [u.a.]

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Please use a persistent id in citations: doi:10.1038/cddis.2011.112

Abstract: Huntington's disease (HD) is a complex and severe disorder characterized by the gradual and the progressive loss of neurons, predominantly in the striatum, which leads to the typical motor and cognitive impairments associated with this pathology. HD is caused by a highly polymorphic CAG trinucleotide repeat expansion in the exon-1 of the gene encoding for huntingtin protein. Since the first discovery of the huntingtin gene, investigations with a consistent number of in-vitro and in-vivo models have provided insights into the toxic events related to the expression of the mutant protein. In this review, we will summarize the progress made in characterizing the signaling pathways that contribute to neuronal degeneration in HD. We will highlight the age-dependent loss of proteostasis that is primarily responsible for the formation of aggregates observed in HD patients. The most promising molecular targets for the development of pharmacological interventions will also be discussed.

Keyword(s): Animals (MeSH) ; Disease Models, Animal (MeSH) ; Humans (MeSH) ; Huntington Disease: metabolism (MeSH) ; Huntington Disease: pathology (MeSH) ; Mice (MeSH) ; Nerve Degeneration: pathology (MeSH)

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Contributing Institute(s):

1. Aging and Neurodegeneration (AG Bano)
2. Animal Facility Bonn (Animal Facility Bonn)
3. Synaptic Connectivity and Neurodegeneration (AG Nicotera)

Research Program(s):

1. 341 - Molecular Signaling (POF3-341) (POF3-341)
2. 342 - Disease Mechanisms and Model Systems (POF3-342) (POF3-342)

Appears in the scientific report 2011

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Database coverage:
; ; ; ; BIOSIS Previews ; Clarivate Analytics Master Journal List ; Current Contents - Life Sciences ; DOAJ Seal ; IF >= 5 ; JCR ; NCBI Molecular Biology Database ; PubMed Central ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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