Small frameshift deletions within the COL4A5 gene in juvenile-onset Alport syndrome

Hum Genet. 1993 Oct;92(4):417-20. doi: 10.1007/BF01247348.

Abstract

Small frameshift deletions within the COL4A5 gene were identified in three Alport syndrome Italian families by non-isotopic single-strand conformation polymorphism (SSCP) screening: in family RMA, a 7-bp deletion (GGGTGAA) in exon 39; in family DGR, a 4-bp deletion (TGGA) in exon 41; in family MIB, deletion of a G in exon 50. The phenotype was characterized by juvenile-onset renal failure with sensorineural hearing loss in males, and a milder clinical pattern in heterozygous females.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Base Sequence
  • Child
  • Chromosome Deletion*
  • DNA / analysis
  • Electrophoresis, Polyacrylamide Gel
  • Exons / genetics
  • Female
  • Frameshift Mutation*
  • Humans
  • Male
  • Middle Aged
  • Molecular Sequence Data
  • Nephritis, Hereditary / genetics*
  • Pedigree
  • Polymerase Chain Reaction
  • Polymorphism, Genetic

Substances

  • DNA