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Journal / Genetics / Differentiation, Maintenance, and Analysis of…
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Genetics

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JoVE Journal Genetics
Differentiation, Maintenance, and Analysis of Human Retinal Pigment Epithelium Cells
 

Differentiation, Maintenance, and Analysis of Human Retinal Pigment Epithelium Cells: A Disease-in-a-dish Model for BEST1 Mutations

Article DOI: 10.3791/57791-v • 06:39 min • August 24th, 2018
August 24th, 2018 •
1, 1, 1, 1
1Department of Pharmacology and Physiology, University of Rochester, School of Medicine and Dentistry

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Summary
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Here we present a protocol to differentiate retinal pigment epithelium (RPE) cells from human pluripotent stem cells bearing patient-derived mutations. The mutant cell lines may be used for functional analyses including immunoblotting, immunofluorescence, and patch clamp. This disease-in-a-dish approach circumvents the difficulty of obtaining native human RPE cells.

Tags

Differentiation Maintenance Analysis Human Retinal Pigment Epithelium Cells Disease-in-a-dish Model BEST1 Mutations Ion-channel Related Retinal Diseases Molecular Mechanisms Bestrophinopathies Therapy RPE Cells Research Purposes Cell Replacement Therapies HPSC Culture Differentiation Medium Human Activin-A Pigmented RPE Cell Clusters Isolate Glass Pasteur Pipettes Microscraper-like Tip
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