Gene targeting of BPAG1: abnormalities in mechanical strength and cell migration in stratified epithelia and neurologic degeneration

Article Abstract:

BPAG1, a 230 kDa protein, is found to be present in the inner plates of the hemidesmosomes, which are specialized integrin-mediated adherens junctions. BPAG1 acts as an anchor for intermediate filaments at the base of the epidermal cells. In absence of BPAG1, there is loss of cell migration and the hemidesmosomes become extremely fragile, lacking an inner plate and attached cytoskeleton. Mechanical integrity is also affected. No loss of cell adhesion or cell growth is seen in mice lacking the BPAG1 gene, but these mice exhibit symptoms of dystonia musculorum (dt/dt mice) in which there is a dystonia accompanied by sensory nerve degeneration.

Genetic aspects, Cell migration, Junctional complexes (Epithelium), Bullous pemphigoid, Dystonia musculorum deformans

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De novo hair follicle morphogenesis and hair tumors in mice expressing a truncated beta-catenin in skin

Article Abstract:

Beta-catenin participates in Wnt signaling, associating with Lef-1/Tcf DNA-binding proteins to produce a transcription factor. This pathway was found to function in keratinocytes and that mice expressing a stabilized beta-catenin regulated by an epidermal promoter undergo a procedure similar to de novo hair morphogenesis. The new follicles produced sebaceous glands and dermal papilla, usually established only in embryogenesis.

Mice, Mice (Rodents), Morphogenesis, Hair follicles

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The ovo gene required for cuticle formation and oogenesis in flies is involved in hair formation and spermatogenesis in mice

Article Abstract:

Genetic parallels have been discovered between mice and flies for epidermal appendage formation and maturation of germ cells. The zinc finger protein svb/ovo is essential for oogenesis and cuticle formation in Drosophila, but it also is necessary for mice to grow hair and form sperm. This gene indicates a phenotype close to the human disorder Bardet-Biedl syndrome.

Skin, Hair, Spermatogenesis

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