Literature DB >> 10923992

Of mice and men: solving the molecular mysteries of Huntington's disease.

P F Shelbourne1.   

Abstract

Recent advances in the manipulation of mouse embryos provide opportunities for the disciplines of neuroscience and molecular genetics to join forces and tackle some previously intractable questions in this area of research. Even Huntington's disease has started to yield clues to its complex pathophysiology as a result of the recent application of transgenic technologies. This short review, while necessarily providing some background clinical information on Huntington's disease, will focus on how modifications of the mouse genome have contributed, and are continuing to contribute, to our understanding of the complex disease process. Such new insights may well turn the hope of developing the first effective treatment for this devastating disease into reality.

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Year:  2000        PMID: 10923992      PMCID: PMC1468102          DOI: 10.1046/j.1469-7580.2000.19640617.x

Source DB:  PubMed          Journal:  J Anat        ISSN: 0021-8782            Impact factor:   2.610


  84 in total

Review 1.  Neurobiology of Huntington's disease.

Authors:  A H Sharp; C A Ross
Journal:  Neurobiol Dis       Date:  1996-02       Impact factor: 5.996

2.  Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.

Authors:  M DiFiglia; E Sapp; K O Chase; S W Davies; G P Bates; J P Vonsattel; N Aronin
Journal:  Science       Date:  1997-09-26       Impact factor: 47.728

Review 3.  Huntington disease: advances in molecular and cell biology.

Authors:  A L Jones; J D Wood; P S Harper
Journal:  J Inherit Metab Dis       Date:  1997-06       Impact factor: 4.982

4.  HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain.

Authors:  M A Kalchman; H B Koide; K McCutcheon; R K Graham; K Nichol; K Nishiyama; P Kazemi-Esfarjani; F C Lynn; C Wellington; M Metzler; Y P Goldberg; I Kanazawa; R D Gietz; M R Hayden
Journal:  Nat Genet       Date:  1997-05       Impact factor: 38.330

5.  Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.

Authors:  S E Browne; A C Bowling; U MacGarvey; M J Baik; S C Berger; M M Muqit; E D Bird; M F Beal
Journal:  Ann Neurol       Date:  1997-05       Impact factor: 10.422

6.  Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo.

Authors:  E Scherzinger; R Lurz; M Turmaine; L Mangiarini; B Hollenbach; R Hasenbank; G P Bates; S W Davies; H Lehrach; E E Wanker
Journal:  Cell       Date:  1997-08-08       Impact factor: 41.582

7.  Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.

Authors:  S W Davies; M Turmaine; B A Cozens; M DiFiglia; A H Sharp; C A Ross; E Scherzinger; E E Wanker; L Mangiarini; G P Bates
Journal:  Cell       Date:  1997-08-08       Impact factor: 41.582

8.  Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

Authors:  I A Klement; P J Skinner; M D Kaytor; H Yi; S M Hersch; H B Clark; H Y Zoghbi; H T Orr
Journal:  Cell       Date:  1998-10-02       Impact factor: 41.582

9.  Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA.

Authors:  P H Reddy; M Williams; V Charles; L Garrett; L Pike-Buchanan; W O Whetsell; G Miller; D A Tagle
Journal:  Nat Genet       Date:  1998-10       Impact factor: 38.330

10.  Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures.

Authors:  P J Skinner; B T Koshy; C J Cummings; I A Klement; K Helin; A Servadio; H Y Zoghbi; H T Orr
Journal:  Nature       Date:  1997-10-30       Impact factor: 49.962

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  1 in total

1.  Modeling Huntington's disease in cells, flies, and mice.

Authors:  S Sipione; E Cattaneo
Journal:  Mol Neurobiol       Date:  2001-02       Impact factor: 5.590

  1 in total

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