Regulation of Myelin-specific gene expression: Relevance to CMT1

John Kamholz*, Raj Awatramani, Daniela Menichella, Huiyuan Jiang, Xu Wenbo, Shy Michael

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

19 Scopus citations


Schwann cells, the myelinating cells of the peripheral nervous system, are derived from the neural crest. Once neural crest cells are committed to the Schwann cell fate, they can take on one of two phenotypes to become myelinating or nonmyelinating Schwann cells, a decision that is determined by interactions with axons. The critical step in the differentiation of myelinating Schwann cells is the establishment of a one-to-one relationship with axons, the so-called "promyelinating" stage of Schwann cell development. The transition from the promyelinating to the myelinating stage of development is then accompanied by a number of significant changes in the pattern of gene expression, including the activation of a set of genes encoding myelin structural proteins and lipid biosynthetic enzymes, and the inactivation of a set of genes expressed only in immature or nonmyelinating Schwann cells. These changes are regulated mainly at the transcriptional level and also require continuous interaction between Schwann cells and their axons.

Original languageEnglish (US)
Pages (from-to)91-108
Number of pages18
JournalAnnals of the New York Academy of Sciences
StatePublished - 1999

ASJC Scopus subject areas

  • Neuroscience(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • History and Philosophy of Science


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