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Altered keratin expression in ichthyosis hystrix Curth-Macklin. A light and electron microscopic study
K M Niemi1, I Virtanen, L Kanerva
1Department of Dermatology, Helsinki University Central Hospital, Finland.
Archives of Dermatological Research
|January 1, 1990
Summary
Ichthyosis hystrix Curth-Macklin involves abnormal keratin gene expression, leading to a skin disorder. This study reveals a pathological expression of fetal keratins, not premature keratinization, in affected skin.
Area of Science:
- Dermatology
- Cell Biology
- Genetics
Background:
- Ichthyosis hystrix Curth-Macklin is a rare ichthyotic disease.
- Understanding its pathogenesis is crucial for diagnosis and treatment.
Purpose of the Study:
- To investigate the pathogenesis of ichthyosis hystrix Curth-Macklin.
- To analyze keratin expression and keratinization processes in affected skin.
Main Methods:
- Immunohistochemistry using monoclonal antibodies (Mabs) against specific keratins.
- Electron microscopy to examine cellular structures in skin biopsies.
Main Results:
- Increased reactivity with Mabs in multiple cell layers, indicating abnormal keratin expression.
- Distinct basal cell layer reaction with Mab PKK1, suggesting fetal keratin expression.
- Electron microscopy revealed tonofilament shells, perinuclear vacuoles, and binuclear keratinocytes.
Conclusions:
- Ichthyosis hystrix Curth-Macklin is characterized by pathological expression of fetal keratins.
- The condition results from abnormal keratin gene expression, not premature keratinization.