1. Allogeneic Cell Therapy for Epidermolysis Bullosa
- Author
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Shasa Hu, Varee Poocheron, and Robert S. Kirsner
- Subjects
medicine.medical_specialty ,Pathology ,Collagen Type VII ,Allogeneic cell ,Cell- and Tissue-Based Therapy ,macromolecular substances ,Dermatology ,Biochemistry ,Molecular level ,Anchoring fibrils ,medicine ,COL7A1 Gene ,Humans ,Transplantation, Homologous ,Basal cell ,Molecular Biology ,integumentary system ,business.industry ,Cell Biology ,Fibroblasts ,medicine.disease ,Cutaneous ulcers ,Type VII collagen ,Epidermolysis bullosa ,Epidermolysis Bullosa ,business - Abstract
Recessive dystrophic epidermolysis bullosa (RDEB) is a severe mechanobullous disease characterized at the molecular level by mutations in the COL7A1 gene, leading to reduced type VII collagen and defective anchoring fibrils at the dermal–epidermal junction (DEJ) (Fine et al., 2000). Patients who inherit this condition suffer from recurrent blistering, cutaneous ulcers, severe scarring, and deformities, and they are at high risk for developing life-threatening squamous cell carcinoma (Mallipeddi, 2002). Currently, there is no treatment for RDEB.
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