Kalinin is abnormally expressed in epithelial basement membranes of Herlitz's junctional epidermolysis bullosa patients.
Kalinin is an extracellular adhesion molecule specific to epithelial basement membranes (BM) identified as a component of anchoring filaments of hemidesmosomes. This heterotrimeric protein is synthesized by cultured normal human keratinocytes and is involved in cell attachment. In indirect immunofluorescence studies, the epidermal BM of patients with junctional epidermolysis bullosa (JEB) of Herlitz's type were found not to be reactive with the anti-kalinin monoclonal antibodies ka146 and K140 and displayed a decreased immunoreactivity to two anti-kalinin antibodies cross-reacting with K-laminin, an anchoring filament component recently described. The intrinsic defect of JEB keratinocytes in the synthesis of kalinin was further documented by indirect immunofluorescence on in vitro cultures of these cells. In non-Herlitz JEB patients, staining of BM was constantly detected. Impairment of expression of kalinin correlated with the lack of reactivity to the monoclonal antibody GB3, which detects the BM component nicein/BM600. These results clearly demonstrate a defect of kalinin expression in epithelial basement membranes of Herlitz JEB patients and suggest that kalinin may play a role in the pathogenesis of the disease. Further studies are in progress to define possible relationships between kalinin and nicein.
Meneguzzi
G
G
Laboratoire de Recherches Dermatologiques, Université de Nice-Sophia Antipolis-06108 Nice, France.
Marinkovich
M P
MP
Aberdam
D
D
Pisani
A
A
Burgeson
R
R
Ortonne
J P
JP
D001485:Basement Membrane / Q000378:metabolism; D015815:Cell Adhesion Molecules / Q000378:metabolism*; D002478:Cells, Cultured; D004817:Epidermis / Q000378:metabolism* / Q000473:pathology; D016109:Epidermolysis Bullosa, Junctional / Q000378:metabolism* / Q000473:pathology; D005333:Fetus; D005455:Fluorescent Antibody Technique; D006801:Humans; D007223:Infant; D015603:Keratinocytes / Q000378:metabolism; D007797:Laminin / Q000378:metabolism; D012016:Reference Values; D000097794:Kalinin
D015815:Cell Adhesion Molecules; D007797:Laminin; D000097794:Kalinin