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Basement membrane proteins kalinin and nicein are structurally and immunologically identical
M P Marinkovich1, P Verrando, D R Keene
1Department of Dermatology, Oregon Health Sciences University, Shriner's Hospital for Crippled Children, Portland.
Laboratory Investigation; a Journal of Technical Methods and Pathology
|September 1, 1993
Summary
Kalinin and nicein are identical proteins involved in skin structure. Both are absent in junctional epidermolysis bullosa (JEB) Herlitz
Area of Science:
- Cell biology
- Dermatology
- Protein biochemistry
Background:
- Junctional epidermolysis bullosa (JEB) Herlitz's disease is a severe skin blistering disorder.
- Anchoring filament proteins are crucial for epidermal adhesion.
- Kalinin and nicein are recently described proteins potentially involved in skin integrity.
Purpose of the Study:
- To compare the recently described proteins kalinin and nicein.
- To determine if kalinin and nicein are identical.
- To further define the structural defect in JEB Herlitz's disease.
Main Methods:
- Utilized monoclonal antibodies against kalinin and nicein for immunofluorescence.
- Performed immunoprecipitation and immunoaffinity purification of proteins.
- Analyzed proteins using SDS-PAGE, Western blotting, V8 protease digestion, and rotary shadowing.
Main Results:
- Kalinin and nicein exhibited identical immunofluorescence staining patterns.
- Biochemical analyses (SDS-PAGE, V8 digestion) showed kalinin and nicein are identical 140 kDa peptides.
- Both kalinin and nicein were absent in JEB Herlitz keratinocyte conditioned medium, unlike K-laminin.
Conclusions:
- Kalinin and nicein are biochemically and immunologically identical.
- The absence of kalinin/nicein in JEB Herlitz's disease keratinocyte cultures supports their role in the disease.
- These findings clarify the molecular defect in JEB Herlitz's disease related to anchoring filament proteins.