Medical College of Wisconsin
CTSICores SearchResearch InformaticsREDCap

Loss of optineurin in vivo results in elevated cell death and alters axonal trafficking dynamics. PLoS One 2014;9(10):e109922

Date

10/21/2014

Pubmed ID

25329564

Pubmed Central ID

PMC4199637

DOI

10.1371/journal.pone.0109922

Scopus ID

2-s2.0-84908045074   14 Citations

Abstract

Mutations in Optineurin have been associated with ALS, glaucoma, and Paget's disease of bone in humans, but little is known about how these mutations contribute to disease. Most of the cellular consequences of Optineurin loss have come from in vitro studies, and it remains unclear whether these same defects would be seen in vivo. To answer this question, we assessed the cellular consequences of Optineurin loss in zebrafish embryos to determine if they showed the same defects as have been described in the in vitro studies. We found that loss of Optineurin resulted in increased cell death, as well as subtle cell morphology, cell migration and vesicle trafficking defects. However, unlike experiments on cells in culture, we found no indication that the Golgi apparatus was disrupted or that NF-κB target genes were upregulated. Therefore, we conclude that in vivo loss of Optineurin shows some, but not all, of the defects seen in in vitro work.

Author List

Paulus JD, Link BA

Author

Brian A. Link PhD Professor in the Cell Biology, Neurobiology and Anatomy department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Amino Acid Sequence
Animals
Apoptosis
Axons
Biological Transport
Cell Cycle Proteins
Cell Movement
Conserved Sequence
Embryo, Nonmammalian
Eye Proteins
Gene Expression Regulation, Developmental
Gene Knockdown Techniques
Humans
Membrane Transport Proteins
Mice
Molecular Sequence Data
Mutation
Neural Crest
Protein Structure, Tertiary
Transcription Factor TFIIIA
Zebrafish
jenkins-FCD Prod-486 e3098984f26de787f5ecab75090d0a28e7f4f7c0