Gentamicin for Junctional Epidermolysis Bullosa

Recruiting Phase 1 Phase 2 Interventional Study
Junctional Epidermolysis Bullosa
No Placebo Group Every participant receives an active treatment — no one gets a placebo. Prior Safety Data This treatment has already been tested in at least one earlier human trial.
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At a Glance
Sex
Any
Trial phase
Phase 1/2
Study type
Interventional
Purpose
Treatment
Participants needed
6 (estimated)
Sponsor
University of Southern California · Other
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About This Trial
Herlitz junctional epidermolysis bullosa (H-JEB), an incurable, fatal, inherited skin disease, is caused by loss-of-function mutations in the LAMA3, LAMB3 or LAMC2 genes, resulting in loss of laminin 332 and poor epidermal-dermal adherence. Eighty percent of H-JEB patients have LAMB3 mutations and about 95% of these are nonsense mutations. The investigators recently demonstrated that gentamicin re…
Trial Locations
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Eligibility Criteria
Inclusion Criteria: 1\. JEB patients with nonsense mutations in the LAMB3 gene in either one or two alleles. Exclusion Criteria: 1. JEB patients who do not have nonsense mutations in the LAMB3 gene in either allele. 2. Pre-existing known auditory impairment. 3. Pre-existing known renal impairment…
Contacts

Mei Chen, Ph.D.

3238650621

chenm@usc.edu

CONTACT

David Woodley, M.D.

3238650956

dwoodley@usc.edu

CONTACT