Epidermal-Specific Defect of GPI Anchor in Pig-a Null Mice Results in Harlequin Ichthyosis-Like Features Mariko Hara-Chikuma, Junji Takeda, Masahito.

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Epidermal-Specific Defect of GPI Anchor in Pig-a Null Mice Results in Harlequin Ichthyosis-Like Features Mariko Hara-Chikuma, Junji Takeda, Masahito Tarutani, Yoshikazu Uchida, Walter M. Holleran, Yoko Endo, Peter M. Elias, Shintaro Inoue Journal of Investigative Dermatology Volume 123, Issue 3, Pages 464-469 (September 2004) DOI: 10.1111/j.0022-202X.2004.23227.x Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

Figure 1 Increased transepidermal water loss (TEWL) and reduced skin conductance in Pig-a null mice. (A) TEWL in dorsal skin of wild-type and Pig-a null mice at 6–8 h after birth (mean±SE, four mice per group); *p<0.01. (B) High-frequency superficial skin surface conductance in dorsal skin of wild-type and Pig-a null mice at 6–8 h after birth (mean±SE, four mice per group); *p<0.01. Journal of Investigative Dermatology 2004 123, 464-469DOI: (10.1111/j.0022-202X.2004.23227.x) Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

Figure 2 Defect of SC lipid lamellar and numerous abnormal lamellar bodies in SG in Pig-a null mice. Lipid lamellar membrane structures examined by electron microscopy with RuO4 postfixation (A, Pig-a null mice; B, wild-type mice). Scale bars=0.1 μm. d=desmosome. (C) Stratum granulosum (SG) in Pig-a null examined by electron microscopy with OsO4 postfixation. Arrowheads point to abnormal LB in which lamellar tructures were not clear; (inset) normal lamellar body of wild-type littermate. Scale bars=0.5 μm, inset=0.1 μm. Journal of Investigative Dermatology 2004 123, 464-469DOI: (10.1111/j.0022-202X.2004.23227.x) Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

Figure 3 Defect of filaggrin monomer in Pig-a null mice. Each lane contains an equal amount (10 μg) of epidermal extracts from Pig-a null (-/-) and wild-type (+/+) littermate. Filaggrin (A), involucrin (B) and loricrin (C) proteins were detected by Western immunoblotting as described under Methods. Presented is a representative immunoblot of four separate experiments. (A). Arrows indicate filaggrin monomer and dimer. (B) Arrow: involucrin. (C) Arrow: loricrin. Journal of Investigative Dermatology 2004 123, 464-469DOI: (10.1111/j.0022-202X.2004.23227.x) Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

Figure 4 Correlation of SC free amino acid (FAA) composition. The defective free amino acid contents were obtained subtracting free amino acid content of Pig-a null from that of wild-type and then converting to amino acid composition (Pig-a null defected FFA: %). The composition in mFG (%) is the amino acid profile of murine filaggrin (Genebank accession #A31488). (A) Wild-type versus mFG; (B) Pig-a null versus mFG; (C) Pig-a null defected FFA versus mFG. Journal of Investigative Dermatology 2004 123, 464-469DOI: (10.1111/j.0022-202X.2004.23227.x) Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions

Figure 5 Reduced PP2A activity in Pig-a null mice. (A) Protein phosphatase (PP) activity in epidermis of Pig-a null and wild-type mice. PP activity was calculated from the rate of dephosphorylation of a radioactive substrate as c.p.m. per minute permicrogram protein in the presence and absence of OA (1 μM), a potent inhibitor of PP2A but not PP1 (mean±SE, five mice per group); *p<0.05. **p<0.01. (B) PP2Ac, catalytic subunit of PP2A, proteins were detected by western immunoblotting as described under Materials and Methods. Equal protein (10 μg) were loaded in a lane. A representative immunoblot from three separate experiments is performed. Journal of Investigative Dermatology 2004 123, 464-469DOI: (10.1111/j.0022-202X.2004.23227.x) Copyright © 2004 The Society for Investigative Dermatology, Inc Terms and Conditions