The gene for Bazex-Dupré-Christol syndrome maps to chromosome Xq
P Vabres1, D Lacombe, L G Rabinowitz
1Research Unit INSERM U 393, Hôpital Necker Enfants-Malades, Paris, France.
Abstract:
Bazex-Dupré-Christol syndrome is an inherited condition with skin cancer predisposition characterized by follicular atrophoderma, hypotrichosis, and early onset of multiple basal cell carcinomas. Previous reports suggested an X-linked mode of inheritance. We therefore performed linkage analysis with microsatellite markers of the X chromosome in three families. We obtained evidence for X-linkage and regional assignment to Xq24-q27 of this syndrome (maximal lod score = 5.26 with a recombination fraction of 0% at the DXS1192 locus). This represents a first step towards the identification of a gene involved in hair follicle development and skin tumor formation.
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