Abstract：
The Myelin Regulator Factor (MYRF) is a master regulator governing myelin formation and maintenance in the central nervous system. The conservation of MYRF across metazoans and its broad tissue expression suggest it has functions extending beyond the well-established role in myelination. Loss of MYRF results in developmental lethality in both invertebrates and vertebrates, and MYRF haploinsufficiency in humans causes MYRF-related Cardiac Urogenital Syndrome, underscoring its importance in animal development; however, these mechanisms are largely unexplored. MYRF, an unconventional transcription factor, begins embedded in the membrane and undergoes intramolecular chaperone mediated trimerization, which triggers self-cleavage, allowing its N-terminal segment with an Ig-fold DNA-binding domain to enter the nucleus for transcriptional regulation. Recent research suggests developmental regulation of cleavage, yet the mechanisms remain enigmatic. While some parts of MYRF\'s structure have been elucidated, others remain obscure, leaving questions about how these motifs are linked to its intricate processing and function.
摘要：
髓鞘调节因子(MYRF)是控制中枢神经系统中髓磷脂形成和维持的主要调节因子。MYRF在后生动物中的保守性及其广泛的组织表达表明，它的功能超出了公认的髓鞘形成作用。MYRF的丧失导致无脊椎动物和脊椎动物的发育致死性。人类MYRF单倍功能不全导致MYRF相关的心脏泌尿生殖道综合征，强调其在动物发育中的重要性；然而，这些机制在很大程度上是未经探索的。MYRF,一种非常规的转录因子,开始嵌入膜中，并经历分子内伴侣介导的三聚，引发自我分裂，允许其具有Ig折叠DNA结合域的N末端片段进入细胞核进行转录调节。最近的研究表明卵裂的发育调控，然而，机制仍然是神秘的。虽然已经阐明了MYRF结构的某些部分，其他人仍然默默无闻，留下了关于这些图案如何与其复杂的处理和功能相关联的问题。
