Stolt C Claus, Lommes Petra, Friedrich Ralf P, Wegner Michael
Institut für Biochemie, Universität Erlangen, Fahrstrasse 17, D-91054 Erlangen, Germany.
Development. 2004 May;131(10):2349-58. doi: 10.1242/dev.01114. Epub 2004 Apr 21.
Development of myelin-forming oligodendrocytes in the central nervous system is dependent on at least two members of the Sox family of high-mobility-group-containing transcription factors. Sox9 is involved in oligodendrocyte specification, whereas Sox10 is required for terminal differentiation. We show that oligodendrocytes in the spinal cord additionally express the highly related Sox8. In Sox8-deficient mice, oligodendrocyte development proceeded normally until birth. However, terminal differentiation of oligodendrocytes was transiently delayed at early postnatal times. Sox8-deficient mice thus exhibited a similar, but less severe phenotype than did Sox10-deficient mice. Terminal oligodendrocyte differentiation was dramatically delayed in Sox8-deficient mice with only a single functional Sox10 allele hinting at redundancy between both Sox proteins. This redundancy was also evident from the fact that Sox8 bound to naturally occurring Sox10 response elements, was able to form DNA-dependent heterodimers with Sox10 and activated Sox10-specific oligodendrocytic target genes in a manner similar to Sox10. However, Sox8 expression levels were significantly lower than those for Sox10. Resulting differences in protein amounts might be a main reason for the weaker impact of Sox8 on oligodendrocyte development and for unidirectional compensation of the Sox8 loss by Sox10.
中枢神经系统中形成髓鞘的少突胶质细胞的发育至少依赖于高迁移率族转录因子Sox家族的两个成员。Sox9参与少突胶质细胞的特化,而Sox10是终末分化所必需的。我们发现脊髓中的少突胶质细胞还额外表达高度相关的Sox8。在Sox8基因缺失的小鼠中,少突胶质细胞的发育在出生前进展正常。然而,少突胶质细胞的终末分化在出生后早期短暂延迟。因此,Sox8基因缺失的小鼠表现出与Sox10基因缺失的小鼠相似但程度较轻的表型。在只有一个功能性Sox10等位基因的Sox8基因缺失的小鼠中,少突胶质细胞的终末分化显著延迟,这暗示了两种Sox蛋白之间的冗余性。这种冗余性也从以下事实中明显看出:Sox8与天然存在的Sox10反应元件结合,能够与Sox10形成DNA依赖性异二聚体,并以与Sox10相似的方式激活Sox10特异性的少突胶质细胞靶基因。然而,Sox8的表达水平明显低于Sox10。由此产生的蛋白量差异可能是Sox8对少突胶质细胞发育影响较弱以及Sox10对Sox8缺失进行单向补偿的主要原因。