Midline axon guidance and human genetic disorders

Clin Genet. 2011 Sep;80(3):226-34. doi: 10.1111/j.1399-0004.2011.01735.x. Epub 2011 Jul 25.

Abstract

In bilaterally symmetric animals, many axons cross the midline to interconnect the left and right sides of the central nervous system (CNS). This process is critical for the establishment of neural circuits that control the proper integration of information perceived by the organism and the resulting response. While neurons at different levels of the CNS project axons across the midline, the molecules that regulate this process are common to many if not all midline-crossing regions. This article reviews the molecules that function as guidance cues at the midline in the developing vertebrate spinal cord, cortico-spinal tract and corpus callosum. As well, we describe the mutations that have been identified in humans that are linked to axon guidance and midline-crossing defects.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Axons / physiology*
  • Corpus Callosum / cytology
  • Corpus Callosum / metabolism*
  • DCC Receptor
  • Gene Expression Regulation, Developmental*
  • Genetics, Medical
  • Humans
  • Mice
  • Mice, Knockout
  • Mutation
  • Nerve Growth Factors / genetics
  • Nerve Growth Factors / metabolism
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism
  • Nervous System Malformations / genetics*
  • Nervous System Malformations / metabolism
  • Nervous System Malformations / pathology
  • Netrin-1
  • Neurogenesis / genetics*
  • Receptors, Cell Surface / genetics
  • Receptors, Cell Surface / metabolism
  • Receptors, Immunologic / genetics
  • Receptors, Immunologic / metabolism
  • Spinal Cord / cytology
  • Spinal Cord / metabolism*
  • Tumor Suppressor Proteins / genetics
  • Tumor Suppressor Proteins / metabolism
  • Vertebrates / genetics
  • Vertebrates / metabolism*

Substances

  • DCC Receptor
  • DCC protein, human
  • Nerve Growth Factors
  • Nerve Tissue Proteins
  • Receptors, Cell Surface
  • Receptors, Immunologic
  • SLIT1 protein, human
  • Tumor Suppressor Proteins
  • roundabout protein
  • Netrin-1

Supplementary concepts

  • Schisis association