Disease: Leber congenital amaurosis
- <em>PNPLA6</em> disorders: what's in a name?
- <em>RPE65</em> mutations in Leber congenital amaurosis, early-onset severe retinal dystrophy, and retinitis pigmentosa from a tertiary eye care center in India
- A combination treatment based on drug repurposing demonstrates mutation-agnostic efficacy in pre-clinical retinopathy models
- A multidisciplinary approach to inherited retinal dystrophies from diagnosis to initial care: a narrative review with inputs from clinical practice
- A novel pathogenic CRB1 variant presenting as Leber Congenital Amaurosis 8 and evaluation of gene editing feasibility
- A Report on Children with CEP290 Mutation, Vision Loss, and Developmental Delay
- A systematic review of inherited retinal dystrophies in Pakistan: updates from 1999 to April 2023
- AAV Serotypes and Their Suitability for Retinal Gene Therapy
- Ablation of Fatty Acid Transport Protein-4 Enhances Cone Survival, M-cone Vision, and Synthesis of Cone-Tropic 9-cis-Retinal in rd12 Mouse Model of Leber Congenital Amaurosis
- An Overview of Nonclinical and Clinical Liver Toxicity Associated With AAV Gene Therapy
- Analysis of CRB1 Pathogenic Variants Correctable with CRISPR Base and Prime Editing
- Application of patient-derived induced pluripotent stem cells and organoids in inherited retinal diseases
- Author Correction: Reduced ADP off-rate by the yeast CCT2 double mutation T394P/R510H which causes Leber congenital amaurosis in humans
- Autosomal Recessive Rod-Cone Dystrophy with Mild Extra-Ocular Manifestations Due to a Splice-Affecting Variant in <em>BBS9</em>
- Autozygome-guided exome-first study in a consanguineous cohort with early-onset retinal disease uncovers an isolated RIMS2 phenotype and a retina-enriched RIMS2 isoform
- Bilateral exudative retinal detachments after subretinal gene therapy with voretigene neparvovec-rzyl for RPE65 Leber Congenital Amaurosis
- Cat LCA-CRX Model, Homozygous for an Antimorphic Mutation Has a Unique Phenotype
- Cell-based Therapies for Corneal and Retinal Disorders
- Central visual pathways affected by degenerative retinal disease before and after gene therapy
- Characteristics of Eyes With CRB1-Associated EOSRD/LCA: Age-Related Changes
- Characterization and AAV-mediated <em>CRB</em> gene augmentation in human-derived <em>CRB1</em><sup>KO</sup> and <em>CRB1</em><sup>KO</sup><em>CRB2</em><sup>+/-</sup> reti
- Classification and Growth Rate of Chorioretinal Atrophy after Voretigene Neparvovec-Rzyl for RPE65-Mediated Retinal Degeneration
- Clinical and Genetic Characterization of RDH12-Retinal Dystrophy in a South American Cohort
- Clinical and genetic studies for a cohort of patients with Leber congenital amaurosis
- Clinical and Molecular Characterization of AIPL1-Associated Leber Congenital Amaurosis/Early-Onset Severe Retinal Dystrophy
- Clinical, Genetic and Histopathological Characteristics of CRX-associated Retinal Dystrophies
- Clinical, genetic, and structural characterization of a novel TUBB4B tubulinopathy
- Clinical, Ophthalmic and Genetic Characterization of RPGRIP1-Associated Leber Congenital Amaurosis/Early Onset Severe Retinal Dystrophy
- Clinical, Ophthalmic, and Genetic Characterization of RPGRIP1-Associated Leber Congenital Amaurosis/Early-Onset Severe Retinal Dystrophy
- Comparison of Worldwide Disease Prevalence and Genetic Prevalence of Inherited Retinal Diseases and Variant Interpretation Considerations
- Compound heterozygous mutations in a mouse model of Leber congenital amaurosis reveal the role of CCT2 in photoreceptor maintenance
- Comprehensive analysis of two hotspot codons in the TUBB4B gene and associated phenotypes
- Comprehensive Genotyping and Phenotyping Analysis of GUCY2D-Associated Rod- and Cone-Dominated Dystrophies
- Correction to: "Gene therapy for retinitis pigmentosa and Leber congenital amaurosis caused by defects in AIPL1: effective rescue of mouse models of partial and complete Aipl1 deficiency using AAV2/2 and AAV2/8 vectors"
- Correction to: Gene therapy for retinitis pigmentosa and Leber congenital amaurosis caused by defects in AIPL1: effective rescue of mouse models of partial and complete Aipl1 deficiency using AAV2/2 and AAV2/8 vectors
- Correction: Development of an optimized AAV2/5 gene therapy vector for Leber congenital amaurosis owing to defects in RPE65
- Could internal limiting membrane peeling before Voretigen neparvovec-ryzl subretinal injection prevent focal chorioretinal atrophy?
- CRB1 is required for recycling by RAB11A+ vesicles in human retinal organoids
- CRB1-associated retinal degeneration is dependent on bacterial translocation from the gut
- CRISPR-Cas9 correction of a nonsense mutation in <em>LCA5</em> rescues lebercilin expression and localization in human retinal organoids
- Cross-species single-cell landscapes identify the pathogenic gene characteristics of inherited retinal diseases
- Current Advancements in Mouse Models of Retinal Disease
- Development of a novel prediction model based on protein structure for identifying RPE65-associated inherited retinal disease (IRDs) of missense variants
- Diagnostic Challenges in <em>ABCA4</em>-Associated Retinal Degeneration: One Gene, Many Phenotypes
- Disease-specific variant interpretation highlighted the genetic findings in 2325 Japanese patients with retinitis pigmentosa and allied diseases
- Durable vision improvement after a single intravitreal treatment with antisense oligonucleotide in <em>CEP2</em>90-LCA: Replication in two eyes
- Effective AAV-mediated gene replacement therapy in retinal organoids modeling AIPL1-associated LCA4
- Electroretinography in congenital nystagmus patients with a normal fundus examination
- Eupatilin Improves Cilia Defects in Human CEP290 Ciliopathy Models
- Expanding the Genotype-Phenotype Correlations and Mutational Spectrum in Inherited Retinal Diseases: Novel and Recurrent Mutations
- Four Unique Genetic Variants in Three Genes Account for 62.7% of Early-Onset Severe Retinal Dystrophy in Chile: Diagnostic and Therapeutic Consequences
- Foveal Hypoplasia in <em>CRB1</em>-Related Retinopathies
- Frequency and Pattern of Worldwide Ocular Gene Therapy Clinical Trials up to 2022
- Gene Augmentation for Autosomal Dominant CRX-Associated Retinopathies
- Gene therapy for heart failure and cardiomyopathies
- Gene Therapy in Hereditary Retinal Dystrophies: The Usefulness of Diagnostic Tools in Candidate Patient Selections
- Generation of Leber congenital amaurosis, type 12 patient-specific induced pluripotent stem cell line (LVPEIi006-A), harboring a homozygous mutation in RD3
- Generation of two hiPSC lines carrying compound heterozygous RDH12 mutations in a LCA patient
- Generation of two induced pluripotent stem cell lines (LVPEIi004-A and LVPEIi005-A) from probands with Leber Congenital Amaurosis 2 (LCA2) and harboring mutations in RPE65
- Genotype-phenotype associations in CRB1 bi-allelic patients: a novel mutation, a systematic review and meta-analysis
- Genotype-Phenotype of CRB1-Associated Early-Onset Retinal Dystrophy: Novel Insights on Retinal Architecture and Therapeutic Window for Clinical Trials
- HIGH MYOPIA IS COMMON IN PATIENTS WITH X-LINKED RETINOPATHIES: Myopic Maculopathy Analysis
- Highly efficient capture approach for the identification of diverse inherited retinal disorders
- Human CRB1 and CRB2 form homo- and heteromeric protein complexes in the retina
- Inherited retinal diseases in Germany-Challenges in health care supply structure and diagnostics
- IQCB1 (NPHP5)-retinopathy: Clinical and Genetic Characterization and Natural History
- Leber congenital amaurosis as the initial and essential manifestation in a Chinese patient with autoimmune polyglandular syndrome Type 1
- Leukemia Inhibitory Factor Protects against Degeneration of Cone Photoreceptors Caused by RPE65 Deficiency
- Long-Term Follow-Up of a Family with Retinal Dystrophy Caused by RPE65 Mutation
- Metabolic changes and retinal remodeling in Heterozygous CRX mutant cats (CRX<sup>RDY/+</sup>)
- Multi-luminance mobility testing after gene therapy in the context of retinal functional diagnostics
- Nonviral base editing of KCNJ13 mutation preserves vision in a model of inherited retinal channelopathy
- Normal vision and development in mice with low functional expression of Kir7.1 in heterozygosis for a blindness-producing mutation inactivating the channel
- Novel Variant <em>IMPDH1</em> c.134A>G, p.(Tyr45Cys): Phenotype-Genotype Correlation Revealed Likely Benign Clinical Significance
- Optical coherence tomography in children with inherited retinal disease
- PAM-flexible Engineered FnCas9 variants for robust and ultra-precise genome editing and diagnostics
- Patient stem cell-derived in vitro disease models for developing novel therapies of retinal ciliopathies
- Phenotyping and genotyping inherited retinal diseases: Molecular genetics, clinical and imaging features, and therapeutics of macular dystrophies, cone and cone-rod dystrophies, rod-cone dystrophies, Leber congenital amaurosis, and cone dysfunction syndro
- Progress and prospects of gene therapy in ophthalmology from 2000 to 2022: A bibliometric analysis
- Publication trends of Leber congenital amaurosis researches: a bibliometric study during 2002-2022
- Qualitative exploration of the visual function impairments and impacts on vision-dependent activities of daily living in Retinitis Pigmentosa and Leber Congenital Amaurosis: content validation of the ViSIO-PRO and ViSIO-ObsRO measures
- Quantification of Fundus Autofluorescence Features in a Molecularly Characterized Cohort of More Than 3000 Inherited Retinal Disease Patients from the United Kingdom
- Real-world experience with Voretigene Neparvovec gene augmentation therapy in RPE65-mutation associated inherited retinal degeneration
- Recent advancements and applications of ophthalmic gene therapy strategies: A breakthrough in ocular therapeutics
- Reduced ADP off-rate by the yeast CCT2 double mutation T394P/R510H which causes Leber congenital amaurosis in humans
- Relationship between genotype, phenotype, and refractive status in patients of inherited retinal degeneration
- RNA-Seq Analysis of Trans-Differentiated ARPE-19 Cells Transduced by AAV9-AIPL1 Vectors
- RPE65 mutations in Leber congenital amaurosis, early-onset severe retinal dystrophy, and retinitis pigmentosa from a tertiary eye care center in India
- Safety and Efficacy of Adeno-Associated Viral Gene Therapy in Patients With Retinal Degeneration: A Systematic Review and Meta-Analysis
- Screening for Autism Spectrum Disorder in Children and Adolescents With Leber's Congenital Amaurosis
- Site-specific genome editing in treatment of inherited diseases: possibility, progress, and perspectives
- Structure of the Ion Channel Kir7.1 and Implications for its Function in Normal and Pathophysiologic States
- The Clinical Findings, Pathogenic Variants, and Gene Therapy Qualifications Found in a Leber Congenital Amaurosis Phenotypic Spectrum Patient Cohort
- The Structural Abnormalities Are Deeply Involved in the Cause of <em>RPGRIP1</em>-Related Retinal Dystrophy in Japanese Patients
- Unique phenotypic-genotypic correlation in Saudi patients with <em>ALMS1</em> mutations
- Unlocking therapeutic potential: dual gene therapy for ameliorating the disease phenotypes in a mouse model of <em>RPE65</em> Leber congenital amaurosis
- Unlocking therapeutic potential: dual gene therapy for ameliorating the disease phenotypes in a mouse model of RPE65 Leber congenital amaurosis
- Update on gene therapies in pediatric ophthalmology
- Voretigene neparvovec for inherited retinal dystrophy due to RPE65 mutations: a scoping review of eligibility and treatment challenges from clinical trials to real practice
- Whole-Exome Sequencing in Turkish Patients with Inherited Retinal Dystrophies Reveals Novel Variants in Ten Genes