Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Prevention of Photoreceptor Cell Loss in a Cln6 Mouse Model of Batten Disease Requires CLN6 Gene Transfer to Bipolar Cells

2 Mar 2018

Abstract excerpt

mice presented mainly with photoreceptor degeneration, supplementation of CLN6 in photoreceptors was not beneficial. Because the level of CLN6 is low in photoreceptors but high in bipolar cells (retinal interneurons that are only lost in Cln6-deficient mice at late disease stages), we explored the therapeutic effects of delivering CLN6 to bipolar cells using adeno-associated virus (AAV) serotype 7m8. Bipolar...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Cellular transport and secretion
Share to Lysosomal Storage Disorders Research
Share to Retinal Development and Disorders

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Source

Identifiers

PMID: 29606505
DOI: 10.1016/j.ymthe.2018.02.027

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 25 Oct 2026.

Related research

Semantic proximity does not establish scientific evidence. Graph snapshot: 2026-09-29.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Prevention of Photoreceptor Cell Loss in a Cln6 Mouse Model of Batten Disease Requires CLN6 Gene Transfer to Bipolar CellsPMID 29606505Read ↗

Gene Therapy Targeting the Inner Retina Rescues the Retinal Phenotype in a Mouse Model of CLN3 Batten Disease.Semantic proximity Read ↗

A mutation in the cone-specific pde6 gene causes rapid cone photoreceptor degeneration in zebrafish.Semantic proximity Read ↗

Therapeutic antisense oligonucleotide mitigates retinal dysfunction in a pig model of CLN3 Batten diseaseSemantic proximity Read ↗

Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons.Semantic proximity Read ↗

Validating Fluorescent Chrnb4.EGFP Mouse Models for the Study of Cone Photoreceptor DegenerationSemantic proximity Read ↗

Molecular Mechanisms Limiting the Therapeutic Window of AAV Gene Therapy in Mouse Models of Blue Cone MonochromacySemantic proximity Read ↗

Neonatal brain-directed gene therapy rescues a mouse model of neurodegenerative CLN6 Batten diseaseSemantic proximity Read ↗

Neonatal brain-directed gene therapy rescues a mouse model of neurodegenerative CLN6 Batten diseaseSemantic proximity Read ↗

Therapeutic Margins in a Novel Preclinical Model of Retinitis PigmentosaSemantic proximity Read ↗

AAV-mediated Gene Therapy Halts Retinal Degeneration in PDE6β-deficient DogsSemantic proximity Read ↗

Wild-type cone photoreceptors persist despite neighboring mutant cone degeneration.Semantic proximity Read ↗

Reduced expression of the nob8 gene does not normalize the distribution or function of mGluR6 in the mouse retina.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.