黑素小体特异性蛋白Pmel17的研究进展  

Melanosomal specific protein Pmel17

作　　者：华优 宋秀祖[1] 

机构地区：[1]安徽医科大学附属杭州临床学院 杭州市第三人民医院皮肤科,杭州310009

出　　处：《国际皮肤性病学杂志》2016年第6期486-489,共4页International Journal of Dermatology and Venereology

基　　金：国家自然科学基金（81000697）;杭州市医学重点专科专病项目（20130733Q24）

摘　　要：Pmel17是黑素小体特异性蛋白,在早期黑素小体中形成纤维性基质,以利于黑素沉积.在Pmel17合成代谢过程中,适配器蛋白与Rab7参与调控其从高尔基体到黑素小体的投递,去整合素金属蛋白酶与pH值参与调控其在黑素小体内形成纤维基质.Pmel17与黑素小体的结构形成,分级组装,转运功能等密切相关,其表达受小眼相关转录因子、眼白化病1型因子、α黑素细胞刺激素等因素的调节.Pmel17参与了白癜风发病,为白癜风的免疫机制研究提供新的方向,Pmel17还可作为黑素瘤的治疗靶点.Pmel17,a melanosomal specific protein,forms fibrous matrices in premelanosomes for the sake of melanin deposition.In the process of Pmel17 synthesis and metabolism,adaptor proteins and Rab7 participate in regulating delivery of Pmel17 from the Golgi apparatus to melanosomes,and the a disintegrin and metalloproteinase （ADAM） family and pH value in regulating formation of fibrous matrices in melanosomes.Pmel17 is closely associated with structure formation,hierarchical assembly and transport of melanosomes,and its expression is regulated by the microphthalmia-associated transcription factor （MITF）,ocular albinism type 1 factor （OA1）,α-melanocyte-stimulating hormone （α-MSH）,etc.Pmel17 is involved in the occurrence of vitiligo,and has provided new directions for studying into the immunological mechanisms of vitiligo.In addition,Pmel17 can serve as a target for the treatment of melanoma.

关 键 词：黑素细胞 白癜风 黑色素瘤 gp100黑色素瘤抗原 黑素小体 Pmel17 

分 类 号：R758.41[医药卫生—皮肤病学与性病学]