Matches in SemOpenAlex for { <https://semopenalex.org/work/W2039111504> ?p ?o ?g. }
- W2039111504 endingPage "35884" @default.
- W2039111504 startingPage "35872" @default.
- W2039111504 abstract "Human diseases caused by mutations in extracellular matrix genes are often associated with an increased risk of cataract and lens capsular rupture. However, the underlying mechanisms of cataract pathogenesis in these conditions are still unknown. Using two different mouse models, we show that the accumulation of collagen chains in the secretory pathway activates the stress signaling pathway termed unfolded protein response (UPR). Transgenic mice expressing ectopic Col4a3 and Col4a4 genes in the lens exhibited activation of IRE1, ATF6, and PERK associated with expansion of the endoplasmic reticulum and attenuation of general protein translation. The expression of the transgenes had adverse effects on lens fiber cell differentiation and eventually induced cell death in a group of transgenic fiber cells. In Col4a1(+/Deltaex40) mutant mice, the accumulation of mutant chains also caused low levels of UPR activation. However, cell death was not induced in mutant lenses, suggesting that low levels of UPR activation are not proapoptotic. Collectively, the results provide in vivo evidence for a role of UPR in cataract formation in response to accumulation of terminally unfolded proteins in the endoplasmic reticulum." @default.
- W2039111504 created "2016-06-24" @default.
- W2039111504 creator A5010301642 @default.
- W2039111504 creator A5040474378 @default.
- W2039111504 creator A5048182525 @default.
- W2039111504 creator A5062427842 @default.
- W2039111504 creator A5074595893 @default.
- W2039111504 creator A5082424779 @default.
- W2039111504 date "2009-12-01" @default.
- W2039111504 modified "2023-10-16" @default.
- W2039111504 title "Abnormal Expression of Collagen IV in Lens Activates Unfolded Protein Response Resulting in Cataract" @default.
- W2039111504 cites W138849845 @default.
- W2039111504 cites W1428480121 @default.
- W2039111504 cites W1546139069 @default.
- W2039111504 cites W1739497080 @default.
- W2039111504 cites W1765185969 @default.
- W2039111504 cites W1851503957 @default.
- W2039111504 cites W1906207528 @default.
- W2039111504 cites W1965606451 @default.
- W2039111504 cites W1970090817 @default.
- W2039111504 cites W1976531450 @default.
- W2039111504 cites W1977982633 @default.
- W2039111504 cites W1978486342 @default.
- W2039111504 cites W1979451483 @default.
- W2039111504 cites W1981171015 @default.
- W2039111504 cites W1984494757 @default.
- W2039111504 cites W1986737010 @default.
- W2039111504 cites W1988482079 @default.
- W2039111504 cites W1988988264 @default.
- W2039111504 cites W1989736688 @default.
- W2039111504 cites W1991999080 @default.
- W2039111504 cites W1994919301 @default.
- W2039111504 cites W1997151300 @default.
- W2039111504 cites W1999868438 @default.
- W2039111504 cites W2005129842 @default.
- W2039111504 cites W2007682828 @default.
- W2039111504 cites W2018269427 @default.
- W2039111504 cites W2023829520 @default.
- W2039111504 cites W2024475665 @default.
- W2039111504 cites W2025846979 @default.
- W2039111504 cites W2028279477 @default.
- W2039111504 cites W2031805491 @default.
- W2039111504 cites W2034810866 @default.
- W2039111504 cites W2039313701 @default.
- W2039111504 cites W2042391634 @default.
- W2039111504 cites W2043387126 @default.
- W2039111504 cites W2045040568 @default.
- W2039111504 cites W2052264293 @default.
- W2039111504 cites W2053271490 @default.
- W2039111504 cites W2055261197 @default.
- W2039111504 cites W2056578856 @default.
- W2039111504 cites W2057969917 @default.
- W2039111504 cites W2060574598 @default.
- W2039111504 cites W2061774676 @default.
- W2039111504 cites W2067540336 @default.
- W2039111504 cites W2068055557 @default.
- W2039111504 cites W2068253591 @default.
- W2039111504 cites W2070273704 @default.
- W2039111504 cites W2072424256 @default.
- W2039111504 cites W2074739656 @default.
- W2039111504 cites W2076880493 @default.
- W2039111504 cites W2083541145 @default.
- W2039111504 cites W2085910719 @default.
- W2039111504 cites W2088375517 @default.
- W2039111504 cites W2088510522 @default.
- W2039111504 cites W2091875431 @default.
- W2039111504 cites W2100297351 @default.
- W2039111504 cites W2106314483 @default.
- W2039111504 cites W2107601804 @default.
- W2039111504 cites W2108317548 @default.
- W2039111504 cites W2113869585 @default.
- W2039111504 cites W2115216273 @default.
- W2039111504 cites W2115763794 @default.
- W2039111504 cites W2116380331 @default.
- W2039111504 cites W2122030028 @default.
- W2039111504 cites W2123872376 @default.
- W2039111504 cites W2127639686 @default.
- W2039111504 cites W2127848114 @default.
- W2039111504 cites W2131210945 @default.
- W2039111504 cites W2131954858 @default.
- W2039111504 cites W2133644385 @default.
- W2039111504 cites W2133771702 @default.
- W2039111504 cites W2134144117 @default.
- W2039111504 cites W2137662702 @default.
- W2039111504 cites W2138009056 @default.
- W2039111504 cites W2142301722 @default.
- W2039111504 cites W2143156113 @default.
- W2039111504 cites W2144065739 @default.
- W2039111504 cites W2147336345 @default.
- W2039111504 cites W2149124155 @default.
- W2039111504 cites W2152522654 @default.
- W2039111504 cites W2165064047 @default.
- W2039111504 cites W2171194384 @default.
- W2039111504 cites W2263583193 @default.
- W2039111504 cites W357311777 @default.
- W2039111504 cites W4242840524 @default.
- W2039111504 cites W4248136323 @default.
- W2039111504 cites W4250374989 @default.