Lamellar Ichthyosis - Genetics

Genetics

This condition is an autosomal recessive genetic disorder, which means the defective gene is located on an autosome, and both parents must carry one copy of the defective gene in order to have a child born with the disorder. Carriers of a recessive gene usually do not show any signs or symptoms of the disorder.

One form of ichthyosis lamellaris (LI1) is associated with a deficiency of the enzyme keratinocyte transglutaminase.

Genes involved include:

Type OMIM Gene Locus
LI1 242300 TGM1 14
LI2 601277 ABCA12 2q34
LI3 604777 CYP4F22 9p13.12
LI5 606545 ? 17p

Read more about this topic:  Lamellar Ichthyosis