Structure and genomic sequence of the myotonic dystrophy (DM kinase) gene

Mani S. Mahadevan, University of Ottawa, Canada
Chris Amemiya, Lawrence Livermore National Laboratory
Gert Jansen, Radboud University Nijmegen
Luc Sabourin, University of Ottawa, Canada
Stephen Baird, Children's Hospital of Eastern Ontario, Ottawa
Catherine E. Neville, University of Ottawa, Canada
Nicole Wormskamp, Radboud University Nijmegen
Bart Segers, Radboud University Nijmegen
Mark Batzer, Lawrence Livermore National Laboratory
Jane Lamerdin, Lawrence Livermore National Laboratory
Peter De Jong, Lawrence Livermore National Laboratory
Bé Wieringa, Radboud University Nijmegen
Robert G. Korneluk, University of Ottawa, Canada

Abstract

The mutation causing myotonic dystrophy (DM) has recently been identified as an unstable CTG trinucleotide repeat located in the 3' untranslated region of a gene encoding for a protein with putative serine-threonine protein kinase activity. In this report we present the genomic sequences of the human and murine DM kinase gene. A comparison of these sequences with each other and with known cDNA sequences from both species, led us to predict a translation initiation codon, as well as determine the organization of the DM kinase gene. Several polymorphisms within the human DM kinase gene have been identified, and PCR assays to detect two of these are described. The complete sequence and characterization of the structure of the DM kinase gene, as well as the identification of novel polymorphisms within the gene, represent an important step in a further understanding of the genetics of myotonic dystrophy and the molecular biology of the gene. © 1993 Oxford University Press.