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| GENETICS AND MOLECULAR-BIOLOGY OF HUNTINGTONS-DISEASE |
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| Author(s): ALBIN RL, TAGLE DA |
| Source: TRENDS IN NEUROSCIENCES Volume: 18 Issue: 1 Pages: 11-14 Published: JAN 1995 |
| Times Cited: 94 References: 54 |
| Abstract: In 1993, the genetic abnormality responsible for Huntington's disease was identified as a trinucleotide-repeat expansion in a novel gene. Much has been Teamed about the molecular genetics of Huntington's disease and the possible effects of the trinucleotide expansion in the development of this disease and other neurological disorders. The Huntington's disease locus is widely expressed throughout the brain and in many non-neural tissues. Current speculation about the pathogenesis of neuronal death concentrates on a 'gain of function' effect in which the abnormal protein has acquired a new and lethal property. Future research will define the normal function of the Huntington's disease locus, test hypotheses regarding the putative gain of function, and explore the factors that determine neuronal susceptibility to the effects of the abnormal allele. |
| Document Type: Article |
| Language: English |
| Reprint Address: ALBIN, RL (reprint author), UNIV MICHIGAN, DEPT NEUROL, ANN ARBOR, MI 48109 USA |
Addresses:
1. NATL CTR HUMAN GENOME RES, GENE TRANSFER LAB, BETHESDA, MD 20892 USA |
| Publisher: ELSEVIER SCI LTD, THE BOULEVARD, LANGFORD LANE, KIDLINGTON, OXFORD, OXON, ENGLAND OX5 1GB |
| Subject Category: Neurosciences |
| IDS Number: QB152 |
| ISSN: 0166-2236 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |