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Spongecake and eggroll: two hereditary diseases in Drosophila resemble patterns of human brain degeneration

Min, Kyung-Tai and Benzer, Seymour (1997) Spongecake and eggroll: two hereditary diseases in Drosophila resemble patterns of human brain degeneration. Current Biology, 7 (11). pp. 885-888. ISSN 0960-9822. https://resolver.caltech.edu/CaltechAUTHORS:20190227-110312751

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Abstract

Various neuronal degenerative diseases are characterized by late onset, relentless progression, and finally death. Many have a direct genetic basis; others are of still unknown etiological mechanisms [1], [2]. The study of human neurodegenerative diseases is complicated by the difficulty of obtaining tissue samples at various stages of progression, especially early in the course of the disease. Since neurodegeneration occurs in many organisms [3], [4], [5], model organisms amenable to genetic and molecular techniques, such as the mouse, offer important advantages. Much less laborious and expensive are worms or flies, which have short generation times and can be rapidly screened for mutations. To investigate the use of the fly as a model system for identifying genes related to such diseases, we screened for mutants having reduced lifespan, then examined them for brain degeneration. We describe here two such mutants, each with a different pattern of degeneration as characterized by light and transmission electron microscopy. The brain of the aging spongecake mutant exhibits regionally specific, membrane-bound vacuoles similar to those seen in spongiform degenerations such as Creutzfeldt-Jakob disease [6], [7]. The mutant eggroll develops dense, multilamellated structures in the brain, resembling ones found in lipid storage diseases such as Tay-Sachs [8].


Item Type:Article
Related URLs:
URLURL TypeDescription
https://doi.org/10.1016/S0960-9822(06)00378-2DOIArticle
Additional Information:© 1997 Elsevier Science Ltd. Received 25 July 1997, Revised 20 August 1997, Accepted 1 September 1997. We thank Rosalind Young and Karen Chang for expert technical support. This research was supported by a fellowship to K-T. M. from the Del Webb foundation and grants to S.B. from the National Institutes of Health (EY 09278 & AG12289), the National Science Foundation (MCB 9408718), the McKnight Foundation, the French Foundation, and the James G. Boswell Foundation.
Funders:
Funding AgencyGrant Number
Del Webb FoundationUNSPECIFIED
NIHEY 09278
NIHAG12289
NSFMCB-9408718
McKnight FoundationUNSPECIFIED
John Douglas French Alzheimer’s FoundationUNSPECIFIED
James G. Boswell FoundationUNSPECIFIED
Issue or Number:11
Record Number:CaltechAUTHORS:20190227-110312751
Persistent URL:https://resolver.caltech.edu/CaltechAUTHORS:20190227-110312751
Official Citation:Kyung-Tai Min, Seymour Benzer, Spongecake and eggroll: two hereditary diseases in Drosophila resemble patterns of human brain degeneration, Current Biology, Volume 7, Issue 11, 1997, Pages 885-888, ISSN 0960-9822, https://doi.org/10.1016/S0960-9822(06)00378-2. (http://www.sciencedirect.com/science/article/pii/S0960982206003782)
Usage Policy:No commercial reproduction, distribution, display or performance rights in this work are provided.
ID Code:93299
Collection:CaltechAUTHORS
Deposited By: Tony Diaz
Deposited On:27 Feb 2019 19:19
Last Modified:03 Oct 2019 20:52

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