Dystroglycan and Mitochondrial Ribosomal Protein L34 Regulate Differentiation in the Drosophila Eye

Hdl Handle:
http://hdl.handle.net/10675.2/584
Title:
Dystroglycan and Mitochondrial Ribosomal Protein L34 Regulate Differentiation in the Drosophila Eye
Authors:
Zhan, Yougen; Melian, Nadia Y.; Pantoja, Mario; Haines, Nicola; Ruohola-Baker, Hannele; Bourque, Charles W.; Rao, Yong; Carbonetto, Salvatore
Abstract:
Mutations that diminish the function of the extracellular matrix receptor Dystroglycan (DG) result in muscular dystrophies, with associated neuronal migration defects in the brain and mental retardation e.g. Muscle Eye Brain Disease. To gain insight into the function of DG in the nervous system we initiated a study to examine its contribution to development of the eye of Drosophila melanogaster. Immuno-histochemistry showed that DG is concentrated on the apical surface of photoreceptors (R) cells during specification of cell-fate in the third instar larva and is maintained at this location through early pupal stages. In point mutations that are null for DG we see abortive R cell elongation during differentiation that first appears in the pupa and results in stunted R cells in the adult. Overexpression of DG in R cells results in a small but significant increase in their size. R cell differentiation defects appear at the same stage in a deficiency line Df(2R)Dg248 that affects Dg and the neighboring mitochondrial ribosomal gene, mRpL34. In the adult, these flies have severely disrupted R cells as well as defects in the lens and ommatidia. Expression of an mRpL34 transgene rescues much of this phenotype. We conclude that DG does not affect neuronal commitment but functions R cell autonomously to regulate neuronal elongation during differentiation in the pupa. We discuss these findings in view of recent work implicating DG as a regulator of cell metabolism and its genetic interaction with mRpL34, a member of a class of mitochondrial genes essential for normal metabolic function.
Editors:
Mei, Lin
Citation:
PLoS One. 2010 May 5; 5(5):e10488
Issue Date:
5-May-2010
URI:
http://hdl.handle.net/10675.2/584
DOI:
10.1371/journal.pone.0010488
PubMed ID:
20463973
PubMed Central ID:
PMC2864756
Type:
Article
ISSN:
1932-6203
Appears in Collections:
Department of Neurology: Faculty Research and Presentations

Full metadata record

DC FieldValue Language
dc.contributor.authorZhan, Yougenen_US
dc.contributor.authorMelian, Nadia Y.en_US
dc.contributor.authorPantoja, Marioen_US
dc.contributor.authorHaines, Nicolaen_US
dc.contributor.authorRuohola-Baker, Hanneleen_US
dc.contributor.authorBourque, Charles W.en_US
dc.contributor.authorRao, Yongen_US
dc.contributor.authorCarbonetto, Salvatoreen_US
dc.contributor.editorMei, Lin-
dc.date.accessioned2012-10-26T16:26:47Z-
dc.date.available2012-10-26T16:26:47Z-
dc.date.issued2010-05-5en_US
dc.identifier.citationPLoS One. 2010 May 5; 5(5):e10488en_US
dc.identifier.issn1932-6203en_US
dc.identifier.pmid20463973en_US
dc.identifier.doi10.1371/journal.pone.0010488en_US
dc.identifier.urihttp://hdl.handle.net/10675.2/584-
dc.description.abstractMutations that diminish the function of the extracellular matrix receptor Dystroglycan (DG) result in muscular dystrophies, with associated neuronal migration defects in the brain and mental retardation e.g. Muscle Eye Brain Disease. To gain insight into the function of DG in the nervous system we initiated a study to examine its contribution to development of the eye of Drosophila melanogaster. Immuno-histochemistry showed that DG is concentrated on the apical surface of photoreceptors (R) cells during specification of cell-fate in the third instar larva and is maintained at this location through early pupal stages. In point mutations that are null for DG we see abortive R cell elongation during differentiation that first appears in the pupa and results in stunted R cells in the adult. Overexpression of DG in R cells results in a small but significant increase in their size. R cell differentiation defects appear at the same stage in a deficiency line Df(2R)Dg248 that affects Dg and the neighboring mitochondrial ribosomal gene, mRpL34. In the adult, these flies have severely disrupted R cells as well as defects in the lens and ommatidia. Expression of an mRpL34 transgene rescues much of this phenotype. We conclude that DG does not affect neuronal commitment but functions R cell autonomously to regulate neuronal elongation during differentiation in the pupa. We discuss these findings in view of recent work implicating DG as a regulator of cell metabolism and its genetic interaction with mRpL34, a member of a class of mitochondrial genes essential for normal metabolic function.en_US
dc.rightsZhan et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.en_US
dc.subjectResearch Articleen_US
dc.subjectCell Biology/Morphogenesis and Cell Biologyen_US
dc.subjectCell Biology/Neuronal and Glial Cell Biologyen_US
dc.subjectDevelopmental Biology/Neurodevelopmenten_US
dc.subjectNeuroscience/Neurobiology of Disease and Regenerationen_US
dc.subjectNeuroscience/Neurodevelopmenten_US
dc.subjectNeurological Disorders/Neuromuscular Diseasesen_US
dc.subjectCell Biology/Extra-Cellular Matrixen_US
dc.titleDystroglycan and Mitochondrial Ribosomal Protein L34 Regulate Differentiation in the Drosophila Eyeen_US
dc.typeArticleen_US
dc.identifier.pmcidPMC2864756en_US
dc.contributor.corporatenameDepartment of Neurology-

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