Ataxin-3 With an Altered Conformation That Exposes the Polyglutamine Domain Is Associated With the Nuclear Matrix

Spinocerebellar ataxia type-3 or Machado-Joseph disease (SCA3/MJD) is a member of the CAG/poly-glutamine repeat disease family. In this family of disorders, a normally polymorphic CAG repeat becomes expanded, resulting in expression of an expanded polyglutamine domain in the disease gene product. Ex...

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Veröffentlicht in:	Human molecular genetics 1999-12, Vol.8 (13), p.2377-2385
Hauptverfasser:	Perez, Matthew K., Paulsonl, Henry L., Pittman, Randall N.
Format:	Artikel
Sprache:	eng
Schlagworte:	Antibodies, Monoclonal Ataxin-3 Biological and medical sciences Blotting, Western Cell Line Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Epitopes Fluorescent Antibody Technique Humans Machado-Joseph Disease - genetics Medical sciences Microscopy, Confocal Nerve Tissue Proteins - chemistry Nerve Tissue Proteins - metabolism Neurology nuclear matrix Nuclear Matrix - metabolism Nuclear Proteins Peptides - chemistry Protein Binding Protein Conformation Protein Structure, Tertiary Repressor Proteins Transfection
Online-Zugang:	Volltext
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