资源描述：
《正常及异常之性别发展》由会员上传分享，免费在线阅读，更多相关内容在学术论文-天天文库。
1、正常及異常之性別發展區分及決定性別：1.基因性別2.性腺性別3.荷爾蒙性別4.內生殖器5.外生殖器6.腦部之性別（CNSorbrainsex）7.後天指派決定之性別（Assignmentorrearingsex）8.性別鑑定或性別表現正常發展建立基因性別受孕性腺分化1.性腺分化發展在5-6週,此時Primodialgermcell移行至gonadalridges如果失敗則造成streakgonad,六週前為Bipotential(indifferent)的性腺(包含germcells,specialepithelial,mesenchymeandmesonephr
2、icduct,internalandsexternalgenitaliaundifferentiated)2.接下來的分化由基因調控睪丸基因決定組（TDFgene）(SRYregionofY,sexdeterminingregionoftheYchromosome)對睪丸分化是必須的,如缺少TDFgene會造成gonadaldysgenesisTDF或SRY蛋白質基因對適當的性腺發展是需要的,3.另外autosomegene(steroidogenicenzymes)也是必須的睪丸的形成：6-7週:Sertolicells,spermatogeniccords,s
3、eminiferoustubules,Leydigcells卵巢的發展：約於晚兩週發生Germcells於皮質層增生卵細胞20週時五到七百萬出生時只剩一到兩百萬,此時的卵細胞為prophaseI11在gonadaldysgenesis(45X)則會有大量的atresia管道系統的分化Ambisexualembryo(八週)暫時並存有Wolfian(mesonephric)及Mullerian(paramesonephric)ducts決定因素：AMH(MIF)及testosterone(由睪丸分泌)＊AMH(anti-mullerianhormone):amemb
4、erofthetransformingfactor-Bfamilyofglycoproteindifferentiationfactorsthatincludesinhibinandactivin.AMH(由Sertolicells合成)八週時可造成單側Mulleriantuberegression抑制oocytemeiosis促使睪丸下降AMH可作為一種serummarker去辦別testiculartissueTestosterone：由Leydig’scells分泌,促使單側wolfianduet分化成epididymus,vasdeferensandsem
5、inalvesicles,ejaculatoryducts局部paracrine作用:adjacentgonad內部的傾向去feminize缺乏AMH-->輸卵管,子宮,陰道上部之發育缺乏testosterone:Wolfiansystem退化外陰部的分化Bipontenal(未分化)stage(6週):neutralprimordial:可發育成男性或女性外生殖器包括Agenitaltubercle,aurogenitalsinus,twolabioscrotalswellings決定因子：由Leydigcells來的Androgen在目標組織以5a-reduc
6、tase把testerone(T)變成dehydrotesterone(DHT)男性化（Masculization):九到十四週MALE:陰莖，陰囊，penileurethra或FEMALE:陰蒂，大陰唇，小陰唇，陰道下段Abnormalandrogeninpact:varibleexternalambiguityCNSdifferentiationAndrogenà編輯CNS男性行為的潛能11性別的指派社會的interactiongenderroleisheavilyinfluencedbyassignmentofsexofrearingfollowedbyso
7、cialinteractionbasedupongenitalappearanceandthedevelopmentofsecondarysexualcharacteristics.異常性別發展A.胎兒內分泌的異常（disordersoffetalendocrinology）Masculinizedfemales(femalepseudohermaphroditism)1.Congenitaladrenalhyperplasia21-hydroxylase(P450c21)deficiency11b-hydroxylase(p450c11b)deficiency3
8、b-hyd
