Function of the neuron-specific alternatively spliced isoforms of nonmuscle myosin II-B during mouse brain development

Mol Biol Cell. 2006 May;17(5):2138-49. doi: 10.1091/mbc.e05-10-0997. Epub 2006 Feb 15.

Abstract

We report that the alternatively spliced isoforms of nonmuscle myosin heavy chain II-B (NHMC II-B) play distinct roles during mouse brain development. The B1-inserted isoform of NMHC II-B, which contains an insert of 10 amino acids near the ATP-binding region (loop 1) of the myosin heavy chain, is involved in normal migration of facial neurons. In contrast, the B2-inserted isoform, which contains an insert of 21 amino acids near the actin-binding region (loop 2), is important for postnatal development of cerebellar Purkinje cells. Deletion of the B1 alternative exon, together with reduced expression of myosin II-B, results in abnormal migration and consequent protrusion of facial neurons into the fourth ventricle. This protrusion is associated with the development of hydrocephalus. Restoring the amount of myosin II-B expression to wild-type levels prevents these defects, showing the importance of total myosin activity in facial neuron migration. In contrast, deletion of the B2 alternative exon results in abnormal development of cerebellar Purkinje cells. Cells lacking the B2-inserted isoform show reduced numbers of dendritic spines and branches. Some of the B2-ablated Purkinje cells are misplaced in the cerebellar molecular layer. All of the B2-ablated mice demonstrated impaired motor coordination.

Publication types

  • Research Support, N.I.H., Intramural

MeSH terms

  • Alternative Splicing*
  • Animals
  • Brain / abnormalities
  • Brain / growth & development*
  • Brain / pathology
  • Cell Movement / genetics
  • Cell Surface Extensions / genetics
  • Cell Surface Extensions / pathology
  • Cerebellum / abnormalities
  • Cerebellum / growth & development
  • Cerebellum / pathology
  • Exons / genetics
  • Facial Nerve / chemistry
  • Facial Nerve / pathology
  • Facial Nerve / physiology
  • Hydrocephalus / genetics*
  • Hydrocephalus / pathology
  • Mice
  • Mice, Mutant Strains
  • Neurons / chemistry
  • Neurons / pathology
  • Neurons / physiology
  • Nonmuscle Myosin Type IIB / analysis
  • Nonmuscle Myosin Type IIB / genetics
  • Nonmuscle Myosin Type IIB / physiology*
  • Protein Isoforms
  • Purkinje Cells / chemistry
  • Purkinje Cells / pathology
  • Purkinje Cells / physiology
  • Sequence Deletion
  • Tissue Distribution
  • Transcription, Genetic

Substances

  • Protein Isoforms
  • Nonmuscle Myosin Type IIB