Amlexanox Enhances Premature Termination Codon Read-Through in COL7A1 and Expression of Full Length Type VII Collagen: Potential Therapy for Recessive Dystrophic Epidermolysis Bullosa.

scientific article published on 23 May 2017

Amlexanox Enhances Premature Termination Codon Read-Through in COL7A1 and Expression of Full Length Type VII Collagen: Potential Therapy for Recessive Dystrophic Epidermolysis Bullosa. is …
instance of (P31):
review articleQ7318358
scholarly articleQ13442814

External links are
P356DOI10.1016/J.JID.2017.05.011
P932PMC publication ID5573171
P698PubMed publication ID28549954

P50authorJosefina Piñón HofbauerQ59689406
John McgrathQ72104357
P2093author name stringAndrew P South
Jouni Uitto
Cristina Has
Leena Bruckner-Tuderman
Mei Chen
Qiujie Jiang
Marco Prisco
Christina Gruber
Velina S Atanasova
P2860cites workFibroblasts show more potential as target cells than keratinocytes in COL7A1 gene therapy of dystrophic epidermolysis bullosaQ45855535
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Targeted Exon Skipping Restores Type VII Collagen Expression and Anchoring Fibril Formation in an In Vivo RDEB ModelQ49132534
High-affinity binding of the NC1 domain of collagen VII to laminin 5 and collagen IV.Q50335736
Collagen VII Half-Life at the Dermal-Epidermal Junction Zone: Implications for Mechanisms and Therapy of Genodermatoses.Q53171208
Injury-Driven Stiffening of the Dermis Expedites Skin Carcinoma Progression.Q53241307
Gentamicin enhanced production of hydrogen peroxide by renal cortical mitochondria.Q53836950
Identification of the glycine-to-arginine substitution G2043R in type VII collagen in a family with dominant dystrophic epidermolysis bullosa from Hungary.Q53924976
Fibroblast-derived dermal matrix drives development of aggressive cutaneous squamous cell carcinoma in patients with recessive dystrophic epidermolysis bullosaQ84077643
Upf1 phosphorylation triggers translational repression during nonsense-mediated mRNA decayQ24323275
Progress toward Treatment and Cure of Epidermolysis Bullosa: Summary of the DEBRA International Research Symposium EB2015Q26775276
NC1 domain of type VII collagen binds to the beta3 chain of laminin 5 via a unique subdomain within the fibronectin-like repeatsQ28297394
A lack of premature termination codon read-through efficacy of PTC124 (Ataluren) in a diverse array of reporter assaysQ28534305
Novel small molecules potentiate premature termination codon readthrough by aminoglycosidesQ28828711
Aminoglycosides restore full-length type VII collagen by overcoming premature termination codons: therapeutic implications for dystrophic epidermolysis bullosa.Q30650123
Inhibition of eukaryotic translation elongation by cycloheximide and lactimidomycinQ33699590
Structural origins of gentamicin antibiotic actionQ33889995
Translational termination efficiency in mammals is influenced by the base following the stop codon.Q34222816
Bone marrow transplantation for recessive dystrophic epidermolysis bullosaQ34256616
An inhibitor of the protein kinases TBK1 and IKK-ɛ improves obesity-related metabolic dysfunctions in miceQ34327061
A post-translational regulatory switch on UPF1 controls targeted mRNA degradationQ34342871
Structural variations in anchoring fibrils in dystrophic epidermolysis bullosa: correlation with type VII collagen expressionQ34362040
Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation systemQ34362841
Olfactory receptor pseudo-pseudogenesQ34543334
Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organsQ34611079
Inherited epidermolysis bullosa: updated recommendations on diagnosis and classificationQ34660939
PDGFRalpha-positive cells in bone marrow are mobilized by high mobility group box 1 (HMGB1) to regenerate injured epitheliaQ34835916
Evidence of abundant stop codon readthrough in Drosophila and other metazoaQ35581562
Glycine substitutions in the triple-helical region of type VII collagen result in a spectrum of dystrophic epidermolysis bullosa phenotypes and patterns of inheritance.Q35881816
Immunohistochemical and mutation analyses demonstrate that procollagen VII is processed to collagen VII through removal of the NC-2 domainQ36382726
Rescue of nonsense mutations by amlexanox in human cellsQ36581500
The E7 gene of human papillomavirus type 16 is sufficient for immortalization of human epithelial cellsQ36688177
Read-through strategies for suppression of nonsense mutations in Duchenne/ Becker muscular dystrophy: aminoglycosides and ataluren (PTC124).Q36907558
Amelioration of epidermolysis bullosa by transfer of wild-type bone marrow cellsQ37082009
Type VII collagen: the anchoring fibril protein at fault in dystrophic epidermolysis bullosaQ37640771
Safety and Wound Outcomes Following Genetically Corrected Autologous Epidermal Grafts in Patients With Recessive Dystrophic Epidermolysis BullosaQ38385390
Rescue of the mucocutaneous manifestations by human cord blood derived nonhematopoietic stem cells in a mouse model of recessive dystrophic epidermolysis bullosaQ39044711
Antisense Oligonucleotide-mediated Exon Skipping as a Systemic Therapeutic Approach for Recessive Dystrophic Epidermolysis BullosaQ39277650
Gene-Corrected Fibroblast Therapy for Recessive Dystrophic Epidermolysis Bullosa using a Self-Inactivating COL7A1 Retroviral VectorQ39904940
Supramolecular interactions in the dermo-epidermal junction zone: anchoring fibril-collagen VII tightly binds to banded collagen fibrilsQ40454600
Restoration of type VII collagen expression and function in dystrophic epidermolysis bullosaQ40690917
SIN retroviral vectors expressing COL7A1 under human promoters for ex vivo gene therapy of recessive dystrophic epidermolysis bullosaQ41811020
Collagen VII plays a dual role in wound healingQ41843053
Intravenously injected recombinant human type VII collagen homes to skin wounds and restores skin integrity of dystrophic epidermolysis bullosaQ41868932
Long-term type VII collagen restoration to human epidermolysis bullosa skin tissueQ41956259
Epidermolysis bullosa and the risk of life-threatening cancers: the National EB Registry experience, 1986-2006.Q42445332
The international dystrophic epidermolysis bullosa patient registry: an online database of dystrophic epidermolysis bullosa patients and their COL7A1 mutations.Q42605404
Correlation between hearing loss and peritonitis frequency and administration of ototoxic intraperitoneal antibiotics in patients with CAPD.Q43144151
P433issue9
P407language of work or nameEnglishQ1860
P921main subjectrecessive dystrophic epidermolysis bullosaQ7302398
P304page(s)1842-1849
P577publication date2017-05-24
P1433published inJournal of Investigative DermatologyQ3186921
P1476titleAmlexanox Enhances Premature Termination Codon Read-Through in COL7A1 and Expression of Full Length Type VII Collagen: Potential Therapy for Recessive Dystrophic Epidermolysis Bullosa
P478volume137

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