Recurrent ctb(7)(q31.3) and possible laminin involvement in a neonatal cutis laxa with a Marfan phenotype

Hum Genet. 1991 Jul;87(3):317-9. doi: 10.1007/BF00200911.

Abstract

A 6-week-old girl presented with cutis laxa, emphysema, heart anomalies and a diaphragmatic hernia. She died at 22 weeks. A recurrent ctb(7)(q31.3) was found and the laminin gene was suspected to be involved in the disease. Anti-human laminin antiserum showed that this protein was absent from the skin. This case, together with 17 other similar cases, could represent a new type of connective tissue disease.

Publication types

  • Case Reports

MeSH terms

  • Cells, Cultured
  • Chromosomes, Human, Pair 7*
  • Cutis Laxa / genetics*
  • Cutis Laxa / pathology
  • Female
  • Humans
  • Immunoenzyme Techniques
  • Infant
  • Karyotyping
  • Laminin / genetics*
  • Marfan Syndrome / genetics*
  • Marfan Syndrome / pathology
  • Phenotype

Substances

  • Laminin