Epidermal transglutaminase in the ichthyoses. |
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Authors: | C A van Hooijdonk P M Steijlen M Bergers P D Mier H Traupe R Happle |
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Affiliation: | Department of Dermatology, University Hospital, Nijmegen, The Netherlands. |
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Abstract: | Membrane-bound transglutaminase (TGm) is responsible for the cross-linking of proteins to form the cornified envelope. Since abnormalities have been reported in the envelope in certain ichthyoses, we have carried out a survey of TGm concentrations in scales from these disorders. Surprisingly, a striking and specific increase in enzyme activity was found in patients with non-erythrodermic autosomal recessive lamellar ichthyosis. It is not clear how this increase is related to the underlying recessive mutation. |
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