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Estrogen insensitivity syndrome : ウィキペディア英語版 | Estrogen insensitivity syndrome
The estrogen insensitivity syndrome (EIS) or estrogen resistance is a form of congenital estrogen deficiency caused by a defective estrogen receptor (ER). Thus, estrogens cannot be recognized and initiate their biological action. In humans, the condition is very rare and only one case has been described. A reported male with EIS was tall as estrogens were unable to act to close the epiphyseal line, at risk for osteoporosis, and sterile (suggesting that in humans estrogens are necessary for reproduction). ==ERKO mice== Estrogen insensitivity syndrome can be experimentally induced in animals, typically mice, by knocking out the estrogen receptor. In so-called ERKO mice different estrogens receptors can be disabled allowing to study the role of such receptors. ERKO mice show development of the respective female or male reproductive systems, and male and female alpha ERKO mice are infertile, beta ERKO males are fertile while females are subfertile, male and female double alpha and beta ERKO mice are sterile. The hypoplastic uterus does not respond to exogenous stimulation by estrogens. Males are infertile with atrophy in the testes. Bones age is delayed and bones are more brittle. Variations in these patterns can be achieved by selectively disabling the alpha or beta ERs.
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