Skewed X-inactivation in cloned mice

Biochem Biophys Res Commun. 2004 Aug 13;321(1):38-44. doi: 10.1016/j.bbrc.2004.06.096.

Abstract

In female mammals, dosage compensation for X-linked genes is accomplished by inactivation of one of two X chromosomes. The X-inactivation ratio (a percentage of the cells with inactivated maternal X chromosomes in the whole cells) is skewed as a consequence of various genetic mutations, and has been observed in a number of X-linked disorders. We previously reported that phenotypically normal full-term cloned mouse fetuses had loci with inappropriate DNA methylation. Thus, cloned mice are excellent models to study abnormal epigenetic events in mammalian development. In the present study, we analyzed X-inactivation ratios in adult female cloned mice (B6C3F1). Kidneys of eight naturally produced controls and 11 cloned mice were analyzed. Although variations in X-inactivation ratio among the mice were observed in both groups, the distributions were significantly different (Ansary-Bradley test, P<0.01). In particular, 2 of 11 cloned mice showed skewed X-inactivation ratios (19.2% and 86.8%). Similarly, in intestine, 1 of 10 cloned mice had a skewed ratio (75.7%). Skewed X-inactivation was observed to various degrees in different tissues of different individuals, suggesting that skewed X-inactivation in cloned mice is the result of secondary cell selection in combination with stochastic distortion of primary choice. The present study is the first demonstration that skewed X-inactivation occurs in cloned animals. This finding is important for understanding both nuclear transfer technology and etiology of X-linked disorders.

MeSH terms

  • Animals
  • Base Sequence
  • Blotting, Southern
  • Brain / physiology
  • Cloning, Molecular
  • Cloning, Organism / methods*
  • DNA / genetics*
  • Databases, Nucleic Acid
  • Female
  • Intestines / physiology
  • Kidney / physiology
  • Lung / physiology
  • Mice
  • Models, Genetic
  • Reference Values
  • Restriction Mapping
  • Sex Chromosome Aberrations*
  • X Chromosome / genetics

Substances

  • DNA