PMC:4502371 / 31103-32168 JSONTXT

{"target":"http://pubannotation.org/docs/sourcedb/PMC/sourceid/4502371","sourcedb":"PMC","sourceid":"4502371","source_url":"https://www.ncbi.nlm.nih.gov/pmc/4502371","text":"We identified dpy-17 mutations as suppressors of mua-3 from two independent genetic screens, suggesting that DPY-17 and MUA-3 genetically interact. DPY-17 encodes a cuticle collagen. C. elegans genome contains approximately 154 collagen genes, many of which are essential and developmental stage-specific (Johnstone 1994, 2000; Kramer 1997). In Marfan syndrome models, the tunica media of the aorta, which normally contains elastin sheets and collagen, is fragmented, disorganized, and lost (Cui et al. 2014). Also, it has been reported that collagen metabolism is abnormal in Marfan patients and overexpression of a collagen gene results in reduction of flexibility of extracellular matrix (ECM), thus contributing to Marfan pathology (Priest et al. 1973; Tajima 1995; Robinson et al. 2006). This might suggest that excessive collagen could negatively affect the structure of ECM when there is not enough Fibrillin-1, and that removal of DPY-17 when mua-3 function is reduced might prevent the formation of abnormal ECM and rescue the lethality during the L4 molt.","tracks":[{"project":"2_test","denotations":[{"id":"25917920-8166670-43299644","span":{"begin":316,"end":320},"obj":"8166670"},{"id":"25917920-10637627-43299645","span":{"begin":322,"end":326},"obj":"10637627"},{"id":"25917920-4582519-43299646","span":{"begin":751,"end":755},"obj":"4582519"},{"id":"25917920-7474647-43299647","span":{"begin":764,"end":768},"obj":"7474647"},{"id":"25917920-16571647-43299648","span":{"begin":786,"end":790},"obj":"16571647"}],"attributes":[{"subj":"25917920-8166670-43299644","pred":"source","obj":"2_test"},{"subj":"25917920-10637627-43299645","pred":"source","obj":"2_test"},{"subj":"25917920-4582519-43299646","pred":"source","obj":"2_test"},{"subj":"25917920-7474647-43299647","pred":"source","obj":"2_test"},{"subj":"25917920-16571647-43299648","pred":"source","obj":"2_test"}]}],"config":{"attribute types":[{"pred":"source","value type":"selection","values":[{"id":"2_test","color":"#ec93da","default":true}]}]}}