Sphingolipid Levels and Processing of the Retinyl Chromophore in the Retina of a Mouse Model of Niemann-Pick Disease
Lysosomal dysfunction and retinal degeneration in ASMase-/- mice are not associated with defects in rod photoreceptor metabolism that affect all-trans retinol formation and availability of NADPH. Lysosomal dysfunction in ASMase-/- mice is not associated with bis-retinoid A2E accumulation.
Authors
- Raf Van de Plas (ORCID: https://orcid.org/0000-0002-2232-7130)
- Bushra Rahman (ORCID: https://orcid.org/0000-0002-3335-3879)
- Masahiro Kono (ORCID: https://orcid.org/0000-0001-7377-1664)
- Lukasz G. Migas (ORCID: https://orcid.org/0000-0002-1884-6405)
- Chunhe Chen
- David M. Anderson (ORCID: https://orcid.org/0000-0002-1049-3158)
- Yiannis Koutalos
- Jian Liu (ORCID: https://orcid.org/0000-0001-9719-2923)
- Kevin L. Schey
- Jie Fan (ORCID: https://orcid.org/0000-0003-0142-9653)
Institutions
- Medical University of South Carolina (US)
- Vanderbilt University (US)
- Delft University of Technology (NL)
Publication Details
- Journal
- Investigative Ophthalmology & Visual Science
- Published
- 2024-12-11
- DOI
- https://doi.org/10.1167/iovs.65.14.24
- Primary Topic
- Lysosomal Storage Disorders Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- University of South Carolina