Sensory deficits in mice hypomorphic for a mammalian homologue of unc-53

Brain Res Dev Brain Res. 2004 Jun 21;150(2):89-101. doi: 10.1016/j.devbrainres.2004.03.004.


The migration of cells and the extension of cellular processes along pathways to their defined destinations are crucial in the development of higher organisms. Caenorhabditis elegans unc-53 plays an important role in cell migration and the outgrowth of cellular processes such as axons. To gain further insight into the biological function of unc53H2, a recently identified mammalian homologue of unc-53, we have generated mice carrying a mutation of unc53H2 and provide evidence that unc53H2 is involved in neuronal development and, more specifically, the development of different sensory systems. The unc53H2 hypomorphic mouse showed a general impaired acuity of several sensory systems (olfactory, auditory, visual and pain sensation) which in case of the visual system was corroborated by the morphological observation of hypoplasia of the optic nerve. We hypothesize that in analogy with its C. elegans homologue, unc53H2 may play a role in the processes of cellular outgrowth and migration.

Publication types

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

MeSH terms

  • Animals
  • Behavior, Animal
  • Blotting, Northern / methods
  • Caenorhabditis elegans Proteins / genetics
  • Caenorhabditis elegans Proteins / physiology*
  • Cloning, Molecular
  • Embryo, Mammalian
  • Embryonic and Fetal Development / physiology*
  • Exploratory Behavior / physiology
  • Female
  • Gene Expression Regulation, Developmental*
  • Genotype*
  • Humans
  • In Situ Hybridization / methods
  • Mice
  • Mice, Inbred Strains
  • Mice, Mutant Strains
  • Microfilament Proteins / genetics
  • Microfilament Proteins / physiology*
  • Motor Activity / genetics
  • Mutation
  • Optic Disk / growth & development
  • Optic Disk / pathology
  • Optic Nerve / growth & development
  • Optic Nerve / pathology
  • Pain / genetics
  • Pain Measurement / methods
  • Pregnancy
  • Psychomotor Performance / physiology
  • RNA, Messenger / biosynthesis
  • Reaction Time / genetics
  • Reflex, Startle / genetics
  • Reverse Transcriptase Polymerase Chain Reaction / methods
  • Rotarod Performance Test / methods
  • Sensation Disorders / genetics*
  • Sequence Homology*


  • Caenorhabditis elegans Proteins
  • Microfilament Proteins
  • RNA, Messenger
  • UNC-53 protein, C elegans