論文 - 詳細
| RRC ID | 3645 |
|---|---|
| 著者 | Winter AD, McCormack G, Page AP. |
| タイトル | Protein disulfide isomerase activity is essential for viability and extracellular matrix formation in the nematode Caenorhabditis elegans. |
| ジャーナル | Dev Biol |
| 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 has been functionally analysed using genetic mutants of the model organism Caenorhabditis elegans. PDI-1 and PDI-3 are individually non-essential, 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 restored following complementation of the mutant with human PDI. |
| 巻・号 | 308(2) |
| ページ | 449-61 |
| 公開日 | 2007-8-15 |
| DOI | 10.1016/j.ydbio.2007.05.041 |
| PII | S0012-1606(07)01111-6 |
| PMID | 17586485 |
| MeSH | Animals Animals, Genetically Modified Caenorhabditis elegans / embryology* Caenorhabditis elegans / enzymology* Caenorhabditis elegans / genetics Caenorhabditis elegans / growth & development Caenorhabditis elegans Proteins / genetics Caenorhabditis elegans Proteins / metabolism Collagen / genetics Collagen / metabolism Evolution, Molecular Extracellular Matrix / enzymology Genes, Helminth Genetic Complementation Test Humans Mutation Phenotype Protein Disulfide-Isomerases / antagonists & inhibitors Protein Disulfide-Isomerases / chemistry Protein Disulfide-Isomerases / genetics Protein Disulfide-Isomerases / metabolism* Protein Structure, Tertiary RNA Interference |
| IF | 2.896 |
| 引用数 | 29 |
| WOS 分野 | DEVELOPMENTAL BIOLOGY |
| オルトメトリクス指標 |
オルトメトリクス指標項目
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| 各媒体での言及数の合計 | 0 |
| 過去6か月間でのオルトメトリクス指標の変動値 | 0.0 |
| リソース情報 | |
| 線虫 | tm0689 |