Fisher A, Caudy M
Department of Cell Biology and Anatomy, Cornell University Medical College, New York, New York, USA.
Bioessays. 1998 Apr;20(4):298-306. doi: 10.1002/(SICI)1521-1878(199804)20:4<298::AID-BIES6>3.0.CO;2-M.
Hairy-related proteins are a distinct subfamily of basic helix-loop-helix (bHLH) proteins that generally function as DNA-binding transcriptional repressors. These proteins act in opposition to bHLH transcriptional activator proteins such as the proneural and myogenic proteins; together, the activator and repressor genes that encode these proteins have co-evolved as a regulatory gene "cassette" or "module" for controlling cell fate decisions. In the development of the Drosophila peripheral nervous system, Hairy-related genes function at multiple steps during neurogenesis, for example, as positional information genes that establish the "prepattern" that controls where "proneural cluster" equivalence groups will form, and later as nuclear effectors of the Notch signaling pathway to "single out" individual precursor cells within the equivalence group. Hairy-related genes also function in the establishment and restriction of other types of equivalence groups, such as those for muscle and Malphigian tubule precursors. This general function in cell fate specification has been conserved from Drosophila to vertebrates and has implications for human disease pathogenesis.
与毛状体相关的蛋白质是碱性螺旋-环-螺旋（bHLH）蛋白质的一个独特亚家族，通常作为DNA结合转录抑制因子发挥作用。这些蛋白质的作用与bHLH转录激活蛋白（如原神经蛋白和生肌蛋白）相反；编码这些蛋白质的激活基因和抑制基因共同进化，形成一个调控基因“盒”或“模块”，用于控制细胞命运决定。在果蝇外周神经系统的发育过程中，与毛状体相关的基因在神经发生的多个步骤中发挥作用，例如，作为位置信息基因，建立控制“原神经簇”等效组形成位置的“前模式”，随后作为Notch信号通路的核效应器，从等效组中“挑选出”单个前体细胞。与毛状体相关的基因也在其他类型等效组的建立和限制中发挥作用，如肌肉和马氏管前体的等效组。这种在细胞命运特化中的一般功能从果蝇到脊椎动物都得到了保留，并且对人类疾病发病机制具有重要意义。
