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Reversing neurodegeneration: a promise unfolds

Article Abstract:

The reversal of neurodegeneration and the work of Rene Hen and colleagues are discussed in this review article. Topics include glutamine repeat diseases and progressive neurodegeneration, and whether impaired protein clearance causes neurodegeneration. A model of reversible neurodegeneration using a line of mice with a transgene with the tetO bidirectional promoter with a lacZ reporter gene on one side and exon1 of huntingtin having 94 CAG repeats (HD94) on the other has been studied. Therapeutic potential is considered.

Author: Orr, Harry T., Zoghbi, Huda Y.
Publisher: Elsevier B.V.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 2000
Usage, Nervous system, Proteins, Cytochemistry, Neurons, Genetically modified mice, Nerve degeneration, Ubiquitin, Spinal cord, Doxycycline, Spinal cord regeneration

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Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinoserebellar ataxia type 1

Article Abstract:

Research has been conducted on spinoserebellar ataxia type 1. The authors report that the phosphatidylinositol 3-kinase/Akt signaling and 14-3-3 modulate the neurotoxicity of the proteins ataxin-1 and provide the insight into spinoserebellar ataxia type 1 pathogenesis.

Author: Orr, Harry T., Zoghbi, Huda Y., Fernandez-Funez, Pedro, Kaytor, Michael D., Chen, Hung-Kai, Acevedo, Summer F., Lam, Yung C., Fernandez, Michael H., Aitken, Alastair, Skoulakis, Efthimios M. C., Botas, Juan
Publisher: Elsevier B.V.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 2003
United Kingdom, Analysis, Causes of, Phosphorylation, Cell research, Cytological research, Pathogenic microorganisms, Nerve proteins, Nerve tissue proteins, Neurodegenerative diseases

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The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/senseless proteins

Article Abstract:

Drosophila Ataxin-1 (dAtx-1), which has conserved AXH domain but lacks a polyglutamine tract is studied to understand how stabilized human Atx-1 induces selective neuronal degeneration. Results show that the Atx-1/Gfi-1 interaction contribute to the selective Purkinje cell degeneration in spinocerebellar ataxia type1 (SCA1).

Author: Orr, Harry T., Zoghbi, Huda Y., Bellen, Hugo J., Botas, Juan, Jafar-Nejad, Hamed, Tsuda, Hirashi, Patel Akash J., Yaling Sun, Hung-Kai Chen, Rose, Matthew F., Venken, Koen J.T.
Publisher: Elsevier B.V.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 2005
Science & research, Bacterial proteins, Genetic research

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Subjects list: Research, United States, Physiological aspects, Genetic aspects, Cell death, Spinocerebellar ataxia
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