Plectin and human genetic disorders of the skin and muscle. The paradigm of epidermolysis bullosa with muscular dystrophy.

@article{Uitto1996PlectinAH,
  title={Plectin and human genetic disorders of the skin and muscle. The paradigm of epidermolysis bullosa with muscular dystrophy.},
  author={Jouni Uitto and Leena Pulkkinen and Frances J. D. Smith and W H Irwin McLean},
  journal={Experimental dermatology},
  year={1996},
  volume={5 5},
  pages={237-46}
}
Recent progress in understanding the molecular organization of the cutaneous basement membrane zone (BMZ) has revealed an intricate network of structural proteins necessary for stable association of the epidermis to the underlying dermis. Molecular genetics of the cutaneous BMZ has also revealed that defects in as many as nine distinct genes within the dermal-epidermal junction which result in different forms of epidermolysis bullosa (EB), a group of heritable mechano-bullous disorders. We have… CONTINUE READING

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