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(American Journal of Pathology. 2002;160:459-468.)
© 2002 American Society for Investigative Pathology

Regular Articles

Defective Laminin 5 Processing in Cylindroma Cells

Lucy Tunggal^*, Juliette Ravaux, Monika Pesch^*, Hans Smola, Thomas Krieg, Françoise Gaill, Takako Sasaki, Rupert Timpl, Cornelia Mauch and Monique Aumailley^*

From the Institute for Biochemistry II^*
and theDepartment of Dermatology,
Medical Faculty,University of Cologne, Cologne, Germany; the Department for ProteinChemistry,
Max-Planck Institute forBiochemistry, Martinsried, Germany; and the Laboratory of MarineBiology,
University Pierre and MarieCurie, Paris, France

Cylindromas are benign skin tumors occurring as multiple nodules characteristically well circumscribed by an excess of basement membrane-like material. To determine the molecular defects leading to extracellular matrix accumulation, the ultrastructural, immunological, and biochemical properties of cylindroma tissue and isolated cells were analyzed. In cylindromas, hemidesmosomes are reduced in number, heterogeneous and immature compared to the normal dermal-epidermal junction. Expression of the 6ß4 integrin in tumor cells is weaker than in basal keratinocytes of the epidermis. Moreover, although in the epidermis 2ß1-integrin expression is restricted to the basal cell layer, it is found in all neoplastic cells within the nodules. Laminin 5 is present throughout the whole thickness of the basement membrane-like zone whereas laminin 10 is restricted to the interface adjacent to the tumor cells. Furthermore, laminin 5 is not properly processed and most of the 3A and 2 laminin chains remain as 165-kd and 155-kd polypeptides, respectively. Mature laminin 5 is thought to be necessary for correct hemidesmosome and basement membrane formation and its abnormal processing, as well as the low expression of 6ß4 integrins, could explain the lack of mature hemidesmosomes. Together, the results show that multiple molecular defects, including alteration of laminin 5 and its integrin receptors, contribute to structural aberrations of the basement membrane and associated structures in cylindromas.

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