Long-Term faithful recapitulation of transglutaminase 1-deficient lamellar ichthyosis in a skin-humanized mouse model, and insights from proteomic studies Articles uri icon

publication date

  • July 2012

start page

  • 1918

end page

  • 1921

issue

  • 7

volume

  • 132

international standard serial number (ISSN)

  • 0022-202X

electronic international standard serial number (EISSN)

  • 1523-1747

abstract

  • The article discusses the association of transglutaminase 1 (TG1)-deficient lamellar ichthyosis (LI) with the mortality rate in the neonatal period. It informs that there is no efficient treatment of the disease and the treatment relieves only some symptoms. It further informs that for the development of the new therapies for LI a stable, preclinical model is required which can recapitulate the phenotype of human skin.

keywords

  • mice lacking; gene