Winter, A. D., McCormack, G. and Page, A. P. (2007) Protein disulfide isomerase activity is essential for viability and extracellular matrix formation in the nematode Caenorhabditis elegans. Developmental Biology, 308(2), pp. 449-461. (doi: 10.1016/j.ydbio.2007.05.041)
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Publisher's URL: http://dx.doi.org/10.1016/j.ydbio.2007.05.041
Abstract
Protein disulfide isomerase (PDI) is a multifunctional protein required for many aspects of protein folding and transit through the endoplasmic reticulum. A conserved family of three PDIs have been functionally analysed using genetic mutants of the model organism Caenorhabditis elegans. PDI-1 and PDI-3 are individually nonessential, whereas PDI-2 is required for normal post-embryonic development. In combination, all three genes are synergistically essential for embryonic development in this nematode. Mutations in pdi-2 result in severe body morphology defects, uncoordinated movement, adult sterility, abnormal molting and aberrant collagen deposition. Many of these phenotypes are consistent with a role in collagen biogenesis and extracellular matrix formation. PDI-2 is required for the normal function of prolyl 4-hydroxylase, a key collagen-modifying enzyme. Site-directed mutagenesis indicates that the independent catalytic activity of PDI-2 may also perform an essential developmental function. PDI-2 therefore performs two critical roles during morphogenesis. The role of PDI-2 in collagen biogenesis can be partially restored following complementation of the mutant with human PDI.
| Item Type: | Articles |
|---|---|
| Status: | Published |
| Refereed: | Yes |
| Glasgow Author(s) Enlighten ID: | Page, Professor Tony and McCormack, Ms Gillian and Winter, Dr Alan |
| Authors: | Winter, A. D., McCormack, G., and Page, A. P. |
| Subjects: | Q Science > QH Natural history > QH345 Biochemistry Q Science > QH Natural history > QH426 Genetics |
| College/School: | College of Medical Veterinary and Life Sciences College of Medical Veterinary and Life Sciences > School of Infection & Immunity |
| Research Group: | Page Group |
| Journal Name: | Developmental Biology |
| Publisher: | Elsevier |
| ISSN: | 0012-1606 |
| ISSN (Online): | 1095-564X |
| Published Online: | 05 June 2007 |
| Copyright Holders: | Copyright © 2007 Elsevier |
| First Published: | First published in Developmental Biology 308(2):449-461 |
| Publisher Policy: | Reproduced in accordance with the copyright policy of the publisher |
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