Journal article
Molecular Genetic Analysis of the Nested Drosophila melanogaster Lamin C Gene
Genetics (Austin), Vol.171(1), pp.185-196
09/2005
DOI: 10.1534/genetics.105.043208
PMCID: PMC1456510
PMID: 15965247
Abstract
Lamins are intermediate filaments that line the inner surface of the nuclear envelope, providing structural support and making contacts with chromatin. There are two types of lamins, A- and B-types, which differ in structure and expression. Drosophila possesses both lamin types, encoded by the\nLamC\n(A-type) and\nlamin Dm\n0\n(B-type) genes.\nLamC\nis nested within an intron of the essential gene\nttv\n. We demonstrate that null mutations in\nLamC\nare lethal, and expression of a wild-type\nLamC\ntransgene rescues lethality of\nLamC\nbut not\nttv\nmutants. Mutations in the human A-type lamin gene lead to diseases called laminopathies. To determine if Drosophila might serve as a useful model to study lamin biology and disease mechanisms, we generated transgenic flies expressing mutant LamC proteins modeled after human disease-causing lamins. These transgenic animals display a nuclear lamin aggregation phenotype remarkably similar to that observed when human mutant A-type lamins are expressed in mammalian cells. LamC aggregates also cause disorganization of lamin Dm\n0\n, indicating interdependence of both lamin types for proper lamina assembly. Taken together, these data provide the first detailed genetic analysis of the\nLamC\ngene and support using Drosophila as a model to study the role of lamins in disease.
Details
- Title: Subtitle
- Molecular Genetic Analysis of the Nested Drosophila melanogaster Lamin C Gene
- Creators
- Sandra R Schulze - Department of Biochemistry, University of Iowa, Iowa City, Iowa 52242Beatrice Curio-Penny - Department of Biochemistry, University of Iowa, Iowa City, Iowa 52242Yuhong Li - Department of Biochemistry, University of Iowa, Iowa City, Iowa 52242Reza A Imani - Department of Biochemistry, University of Iowa, Iowa City, Iowa 52242Lena Rydberg - Department of Biochemistry, University of Iowa, Iowa City, Iowa 52242Pamela K Geyer - Department of Biochemistry, University of Iowa, Iowa City, Iowa 52242Lori L Wallrath - Department of Biochemistry, University of Iowa, Iowa City, Iowa 52242
- Resource Type
- Journal article
- Publication Details
- Genetics (Austin), Vol.171(1), pp.185-196
- DOI
- 10.1534/genetics.105.043208
- PMID
- 15965247
- PMCID
- PMC1456510
- ISSN
- 0016-6731
- eISSN
- 1943-2631
- Language
- English
- Date published
- 09/2005
- Academic Unit
- Obstetrics and Gynecology; Biochemistry and Molecular Biology; University College Courses
- Record Identifier
- 9984024419702771
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