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| Huntington and DRPLA proteins selectively interact with the enzyme GAPDH |
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| Author(s): Burke JR, Enghild JJ, Martin ME, Jou YS, Myers RM, Roses AD, Vance JM, Strittmatter WJ |
| Source: NATURE MEDICINE Volume: 2 Issue: 3 Pages: 347-350 Published: MAR 1996 |
| Times Cited: 319 References: 32 |
| Abstract: At least five adult-onset neurodegenerative diseases, including Huntington disease (HD), and dentatorubral-pallidoluysian atrophy (DRPLA) are produced by genes containing a variably increased CAG repeat within the coding region(1-4). The size range of the repeats is similar in all diseases; unaffected individuals have fewer than 30 CAG repeats, whereas affected patients usually have more than 40 repeats. The size of the inherited CAG repeat correlates with the severity and age of disease onset(1,5-7). The CAG triplet repeat produces a polyglutamine domain in the expressed proteins(3,8-10). All of these diseases are inherited in a dominant fashion, and a pathologic gain of function in gene carriers has been proposed. We sought to identify proteins in the brain that selectively interact with polyglutamine domain proteins, hypothesizing that the polyglutamine domain may determine protein-protein interactions. |
| Document Type: Article |
| Language: English |
| Reprint Address: Burke, JR (reprint author), DUKE UNIV, MED CTR, DEPT MED NEUROL, DURHAM, NC 27710 USA |
Addresses:
1. DUKE UNIV, MED CTR, DEPT PATHOL, DURHAM, NC 27710 USA
2. DUKE UNIV, MED CTR, DEPT NEUROBIOL, DURHAM, NC 27710 USA
3. DUKE UNIV, MED CTR, DEPT GENET, DURHAM, NC 27710 USA
4. DUKE UNIV, MED CTR, DEANE LAB, DURHAM, NC 27710 USA
5. STANFORD UNIV, SCH MED, DEPT GENET, STANFORD, CA 94305 USA |
| Publisher: NATURE PUBLISHING CO, 345 PARK AVE SOUTH, NEW YORK, NY 10010-1707 |
| Subject Category: Biochemistry & Molecular Biology; Cell Biology; Medicine, Research & Experimental |
| IDS Number: TY640 |
| ISSN: 1078-8956 |
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