review article | Q7318358 |
scholarly article | Q13442814 |
P50 | author | Josefina Piñón Hofbauer | Q59689406 |
John Mcgrath | Q72104357 | ||
P2093 | author name string | Andrew P South | |
Jouni Uitto | |||
Cristina Has | |||
Leena Bruckner-Tuderman | |||
Mei Chen | |||
Qiujie Jiang | |||
Marco Prisco | |||
Christina Gruber | |||
Velina S Atanasova | |||
P2860 | cites work | Fibroblasts show more potential as target cells than keratinocytes in COL7A1 gene therapy of dystrophic epidermolysis bullosa | Q45855535 |
Mechanism of the action of amoxanox (AA-673), an orally active antiallergic agent | Q46876547 | ||
The COL7A1 mutation database. | Q48643475 | ||
Targeted Exon Skipping Restores Type VII Collagen Expression and Anchoring Fibril Formation in an In Vivo RDEB Model | Q49132534 | ||
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 bullosa | Q84077643 | ||
Upf1 phosphorylation triggers translational repression during nonsense-mediated mRNA decay | Q24323275 | ||
Progress toward Treatment and Cure of Epidermolysis Bullosa: Summary of the DEBRA International Research Symposium EB2015 | Q26775276 | ||
NC1 domain of type VII collagen binds to the beta3 chain of laminin 5 via a unique subdomain within the fibronectin-like repeats | Q28297394 | ||
A lack of premature termination codon read-through efficacy of PTC124 (Ataluren) in a diverse array of reporter assays | Q28534305 | ||
Novel small molecules potentiate premature termination codon readthrough by aminoglycosides | Q28828711 | ||
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 lactimidomycin | Q33699590 | ||
Structural origins of gentamicin antibiotic action | Q33889995 | ||
Translational termination efficiency in mammals is influenced by the base following the stop codon. | Q34222816 | ||
Bone marrow transplantation for recessive dystrophic epidermolysis bullosa | Q34256616 | ||
An inhibitor of the protein kinases TBK1 and IKK-ɛ improves obesity-related metabolic dysfunctions in mice | Q34327061 | ||
A post-translational regulatory switch on UPF1 controls targeted mRNA degradation | Q34342871 | ||
Structural variations in anchoring fibrils in dystrophic epidermolysis bullosa: correlation with type VII collagen expression | Q34362040 | ||
Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation system | Q34362841 | ||
Olfactory receptor pseudo-pseudogenes | Q34543334 | ||
Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organs | Q34611079 | ||
Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification | Q34660939 | ||
PDGFRalpha-positive cells in bone marrow are mobilized by high mobility group box 1 (HMGB1) to regenerate injured epithelia | Q34835916 | ||
Evidence of abundant stop codon readthrough in Drosophila and other metazoa | Q35581562 | ||
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 domain | Q36382726 | ||
Rescue of nonsense mutations by amlexanox in human cells | Q36581500 | ||
The E7 gene of human papillomavirus type 16 is sufficient for immortalization of human epithelial cells | Q36688177 | ||
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 cells | Q37082009 | ||
Type VII collagen: the anchoring fibril protein at fault in dystrophic epidermolysis bullosa | Q37640771 | ||
Safety and Wound Outcomes Following Genetically Corrected Autologous Epidermal Grafts in Patients With Recessive Dystrophic Epidermolysis Bullosa | Q38385390 | ||
Rescue of the mucocutaneous manifestations by human cord blood derived nonhematopoietic stem cells in a mouse model of recessive dystrophic epidermolysis bullosa | Q39044711 | ||
Antisense Oligonucleotide-mediated Exon Skipping as a Systemic Therapeutic Approach for Recessive Dystrophic Epidermolysis Bullosa | Q39277650 | ||
Gene-Corrected Fibroblast Therapy for Recessive Dystrophic Epidermolysis Bullosa using a Self-Inactivating COL7A1 Retroviral Vector | Q39904940 | ||
Supramolecular interactions in the dermo-epidermal junction zone: anchoring fibril-collagen VII tightly binds to banded collagen fibrils | Q40454600 | ||
Restoration of type VII collagen expression and function in dystrophic epidermolysis bullosa | Q40690917 | ||
SIN retroviral vectors expressing COL7A1 under human promoters for ex vivo gene therapy of recessive dystrophic epidermolysis bullosa | Q41811020 | ||
Collagen VII plays a dual role in wound healing | Q41843053 | ||
Intravenously injected recombinant human type VII collagen homes to skin wounds and restores skin integrity of dystrophic epidermolysis bullosa | Q41868932 | ||
Long-term type VII collagen restoration to human epidermolysis bullosa skin tissue | Q41956259 | ||
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 | ||
P433 | issue | 9 | |
P407 | language of work or name | English | Q1860 |
P921 | main subject | recessive dystrophic epidermolysis bullosa | Q7302398 |
P304 | page(s) | 1842-1849 | |
P577 | publication date | 2017-05-24 | |
P1433 | published in | Journal of Investigative Dermatology | Q3186921 |
P1476 | title | Amlexanox Enhances Premature Termination Codon Read-Through in COL7A1 and Expression of Full Length Type VII Collagen: Potential Therapy for Recessive Dystrophic Epidermolysis Bullosa | |
P478 | volume | 137 |
Q55497000 | Advances in therapeutic use of a drug-stimulated translational readthrough of premature termination codons. |
Q91630917 | Assessment of quality and consistency of monoclonal antibodies for CB1 and CB2 in head and neck squamous cell carcinoma |
Q92368463 | Epidermolysis bullosa: Advances in research and treatment |
Q64945886 | Gentamicin induces LAMB3 nonsense mutation readthrough and restores functional laminin 332 in junctional epidermolysis bullosa. |
Q91652413 | Germline CRISPR/Cas9-Mediated Gene Editing Prevents Vision Loss in a Novel Mouse Model of Aniridia |
Q64286072 | Newer treatment modalities in epidermolysis bullosa |
Q93182236 | PXE, a Mysterious Inborn Error Clarified |
Q89860914 | Succinic Semialdehyde Dehydrogenase Deficiency: An Update |