Journal article
The role of Drosophila Lamin C in muscle function and gene expression
Development (Cambridge), Vol.137(18), pp.3067-3077
09/15/2010
DOI: 10.1242/dev.048231
PMCID: PMC2926956
PMID: 20702563
Abstract
The inner side of the nuclear envelope (NE) is lined with lamins, a meshwork of intermediate filaments that provides structural support for the nucleus and plays roles in many nuclear processes. Lamins, classified as A- or B-types on the basis of biochemical properties, have a conserved globular head, central rod and C-terminal domain that includes an Ig-fold structural motif. In humans, mutations in A-type lamins give rise to diseases that exhibit tissue-specific defects, such as Emery-Dreifuss muscular dystrophy.
Drosophila
is being used as a model to determine tissue-specific functions of A-type lamins in development, with implications for understanding human disease mechanisms. The GAL4-UAS system was used to express wild-type and mutant forms of Lamin C (the presumed
Drosophila
A-type lamin), in an otherwise wild-type background. Larval muscle-specific expression of wild type
Drosophila
Lamin C caused no overt phenotype. By contrast, larval muscle-specific expression of a truncated form of Lamin C lacking the N-terminal head (Lamin C ΔN) caused muscle defects and semi-lethality, with adult ‘escapers’ possessing malformed legs. The leg defects were due to a lack of larval muscle function and alterations in hormone-regulated gene expression. The consequences of Lamin C association at a gene were tested directly by targeting a Lamin C DNA-binding domain fusion protein upstream of a reporter gene. Association of Lamin C correlated with localization of the reporter gene at the nuclear periphery and gene repression. These data demonstrate connections among the
Drosophila
A-type lamin, hormone-induced gene expression and muscle function.
Details
- Title: Subtitle
- The role of Drosophila Lamin C in muscle function and gene expression
- Creators
- George Dialynas - Department of Biochemistry, University of IowaSean Speese - Department of Neurobiology, University of MassachusettsVivian Budnik - Department of Neurobiology, University of MassachusettsPamela K Geyer - Department of Biochemistry, University of IowaLori L Wallrath - Department of Biochemistry, University of Iowa
- Resource Type
- Journal article
- Publication Details
- Development (Cambridge), Vol.137(18), pp.3067-3077
- DOI
- 10.1242/dev.048231
- PMID
- 20702563
- PMCID
- PMC2926956
- NLM abbreviation
- Development
- ISSN
- 0950-1991
- eISSN
- 1477-9129
- Publisher
- Company of Biologists
- Language
- English
- Date published
- 09/15/2010
- Academic Unit
- Obstetrics and Gynecology; Biochemistry and Molecular Biology; University College Courses
- Record Identifier
- 9984024565102771
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