Epidermolysis Bullosa: Prospects for Cell-Based Therapies
نویسندگان
چکیده
منابع مشابه
Stem-cell Based Therapies for Epidermolysis Bullosa
Direct reprogramming of somatic cells into induced pluripotent stem cells (iPSCs) provides an opportunityto develop novel personalized treatment options for numerous diseases and to advance current approachesfor cell-based drug discoveries and disease modeling. The ability to differentiate iPSCs into relevant celltypes is an important prerequisite for the successful development of i...
متن کاملFibroblast-based cell therapy strategy for recessive dystrophic epidermolysis bullosa.
Dystrophic epidermolysis bullosa (DEB) is a severe skin fragility disorder associated with trauma-induced blistering, progressive soft tissue scarring, and increased risk of skin cancer. DEB is caused by mutations in the COL7A1 gene which result in reduced, truncated, or absent type VII collagen, and anchoring fibrils at the dermal-epidermal junction (DEJ). Because no topical wound-healing agen...
متن کاملEpidemiology of epidermolysis bullosa in the antipodes: the Australasian Epidermolysis Bullosa Registry with a focus on Herlitz junctional epidermolysis bullosa.
OBJECTIVE To present epidemiologic and clinical data from the Australasian Epidermolysis Bullosa (EB) Registry, the first orphan disease registry in Australia. DESIGN Observational study (cross-sectional and longitudinal). SETTING Australian private dermatology practice, inpatient ward, and outpatient clinic. PATIENTS Systematic case finding of patients with EB simplex, junctional EB (JEB...
متن کاملEpidermolysis bullosa acquisita.
Autoimmunity to type-WI collagen is characterized by autoantibodies predominantly of lgG class to the non-collagenous domain of type-WI collagen present in the anchoring fibrils which bind basement membrane lamina densa to the anchoring plaques in the dermis. This results in a sublami.na densa split with a blister formation. Type WI collagen autoim.munity is heterogenous in its clinical spectru...
متن کاملJunctional epidermolysis bullosa.
Epidermolysis bullosa (EB) encompasses a heterogeneous group of genodermatoses, characterized by fragility and blistering of the skin, often associated with extracutaneous manifestations. The level of vesiculation within the skin defines 3 major subtypes of EB: EB simplex, junctional EB, and dystrophic EB. We present the case of a male neonate of 36 weeks of gestation, who was born with a few b...
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ژورنال
عنوان ژورنال: Journal of Investigative Dermatology
سال: 2008
ISSN: 0022-202X
DOI: 10.1038/jid.2008.216