Duchenne muscular dystrophy involving translocation of the dmd gene next to ribosomal RNA genes.

Duchenne muscular dystrophy (DMD) is a severe X-linked disorder leading to early death of affected males. Females with the disease are rare, but seven are known to be affected because of a chromosomal rearrangement involving a site at or near the dmd gene on the X chromosome. One of the seven has a translocation between the X and chromosome 21. The translocation-derived chromosomes from this patient have been isolated, and the translocation is shown to have split the block of genes encoding ribosomal RNA on the short arm of chromosome 21. Thus ribosomal RNA gene probes may be used to identify a junction fragment from the translocation site, allowing access to cloned segments of the X at or near the dmd gene and presenting a new approach to the study of this disease.

[1]  E. Zackai,et al.  Further evidence for Xp21 location of Duchenne muscular dystrophy (DMD) locus: X;9 translocation in a female with DMD. , 1983, Journal of medical genetics.

[2]  K. Davies,et al.  Linkage analysis of two cloned DNA sequences flanking the Duchenne muscular dystrophy locus on the short arm of the human X chromosome. , 1983, Nucleic acids research.

[3]  H. Willard,et al.  Isolation and characterization of a major tandem repeat family from the human X chromosome. , 1983, Nucleic acids research.

[4]  K. Davies,et al.  Linkage relationship of a cloned DNA sequence on the short arm of the X chromosome to Duchenne muscular dystrophy , 1982, Nature.

[5]  J. Erickson,et al.  Structure and variation of human ribosomal DNA: the external transcribed spacer and adjacent regions. , 1982, American journal of human genetics.

[6]  M. Zatz,et al.  Translocation (X;6) in a female with Duchenne muscular dystrophy: implications for the localisation of the DMD locus. , 1981, Journal of medical genetics.

[7]  J. Erickson,et al.  Structure and variation of human ribosomal DNA: molecular analysis of cloned fragments. , 1981, Gene.

[8]  P. Hunt,et al.  Duchenne muscular dystrophy (DMD) in a female with an X/autosome translocation: further evidence that the DMD locus is at Xp21. , 1981, American journal of human genetics.

[9]  R. Greenstein,et al.  An (X;11) translocation in a girl with Duchenne muscular dystrophy. Repository identification No. GM1695. , 1980, Cytogenetics and cell genetics.

[10]  G. Clarke,et al.  Muscular dystrophy in an X; 1 translocation female suggests that Duchenne locus is on X chromosome short arm. , 1979, Journal of medical genetics.

[11]  I. Dawid,et al.  Isolation and sequence organization of human ribosomal DNA. , 1979, Journal of molecular biology.

[12]  F. Ruddle,et al.  Genetic analysis of the human cell surface: antigenic marker for the human X chromosome in human-mouse hybrids. , 1978, Proceedings of the National Academy of Sciences of the United States of America.

[13]  R. Worton,et al.  Chromosome loss is responsible for segregation at theHPRT locus in Chinese hamster cell hybrids , 1977, Somatic cell genetics.

[14]  R. Schmickel,et al.  Characterization and Localization of the Human Genes for Ribosomal Ribonucleic Acid , 1977, Pediatric Research.

[15]  E. Southern Detection of specific sequences among DNA fragments separated by gel electrophoresis. , 1975, Journal of molecular biology.

[16]  U. Schibler,et al.  Changes in size and secondary structure of the ribosomal transcription unit during vertebrate evolution. , 1975, Journal of molecular biology.