title: Targeted deletion of the sciellin gene resulted in normal development and maturation.
abstract: Sciellin, together with other precursor proteins, was cross-linked by transglutaminase 1 to form the cornified envelope, an essential component of the physical barrier of the epidermis and stratified squamous epithelia. To more fully understand the function of sciellin in cornified envelope formation, we generated sciellin null mice. The mice appeared normal in their development and maturation and there were no structural features that distinguished them from littermate controls. Isolated cornified envelopes appeared normal in structure and were not more fragile to mechanical stress. There was no evidence of decreased barrier function or altered expression of other cornified envelope components. Transgenic mice expressing the repeat domain appeared to have a normal phenotype, like the null, and did not alter endogenous sciellin expression. We conclude that sciellin null mice had no structural anomalies and the transgenic mice did not act as a dominant-negative mutation.
authors: Department of Dermatology, Cutaneous Biology Research Center, Harvard Medical School, Massachusetts General Hospital, Boston, Massachusetts 02129, USA. hbaden@partners.org
authors: Howard P
authors: HP
authors: Baden
authors: Marie-France
authors: MF
authors: Champliaud
authors: John P
authors: JP
authors: Sundberg
authors: Alain
authors: A
authors: Viel
mesh_terms: D000818:Animals; D002352:Carrier Proteins / Q000235:genetics*; D002454:Cell Differentiation; D002678:Chimera; D004817:Epidermis / Q000254:growth & development*; D017353:Gene Deletion; D015870:Gene Expression; D018390:Gene Targeting; D048788:Growth and Development / Q000235:genetics; D006720:Homozygote; D008565:Membrane Proteins / Q000378:metabolism; D051379:Mice; D008822:Mice, Transgenic / Q000235:genetics / Q000254:growth & development*; D020411:Oligonucleotide Array Sequence Analysis; D010641:Phenotype; D011498:Protein Precursors / Q000378:metabolism; D012867:Skin / Q000166:cytology / Q000196:embryology; D015854:Up-Regulation
chemical_list: D002352:Carrier Proteins; D008565:Membrane Proteins; D011498:Protein Precursors; C111281:SCEL protein, human; C064554:loricrin; C032253:involucrin