Malformation of the Posterior Cerebellar Vermis Is a Common Neuroanatomical Phenotype of Genetically Engineered Mice on the C57BL/6 Background

Cerebellum. 2018 Apr;17(2):173-190. doi: 10.1007/s12311-017-0892-3.

Abstract

C57BL/6 mice exhibit spontaneous cerebellar malformations consisting of heterotopic neurons and glia in the molecular layer of the posterior vermis, indicative of neuronal migration defect during cerebellar development. Recognizing that many genetically engineered (GE) mouse lines are produced from C57BL/6 ES cells or backcrossed to this strain, we performed histological analyses and found that cerebellar heterotopia were a common feature present in the majority of GE lines on this background. Furthermore, we identify GE mouse lines that will be valuable in the study of cerebellar malformations including diverse driver, reporter, and optogenetic lines. Finally, we discuss the implications that these data have on the use of C57BL/6 mice and GE mice on this background in studies of cerebellar development or as models of disease.

Keywords: C57BL/6; Cerebellar development; Knock-out mice; Transgenic mice.

MeSH terms

  • Animals
  • Animals, Newborn
  • Cerebellar Vermis / abnormalities*
  • Cerebellar Vermis / pathology
  • Female
  • Hypoxanthine Phosphoribosyltransferase / genetics
  • Hypoxanthine Phosphoribosyltransferase / metabolism
  • Luminescent Proteins / genetics
  • Luminescent Proteins / metabolism
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Transgenic / physiology*
  • Nervous System Malformations / genetics*
  • Nervous System Malformations / pathology*
  • Receptor, TIE-2 / genetics
  • Receptor, TIE-2 / metabolism
  • Receptors, LDL / genetics
  • Receptors, LDL / metabolism
  • Synaptosomal-Associated Protein 25 / genetics
  • Synaptosomal-Associated Protein 25 / metabolism

Substances

  • Luminescent Proteins
  • Receptors, LDL
  • Synaptosomal-Associated Protein 25
  • Hypoxanthine Phosphoribosyltransferase
  • Receptor, TIE-2
  • Tek protein, mouse