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JoVE Journal Genetics
Differentiering, vedligeholdelse og analyse af menneskers retinale Pigment epitel celler
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Differentiering, vedligeholdelse og analyse af menneskers retinale Pigment epitel celler: en sygdom i skål Model for BEST1 mutationer

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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Her præsenterer vi en protokol for at differentiere retinale pigment epitel (ÅV) celler fra humane pluripotente stamceller forsynet med patienten-afledte mutationer. De mutante cellelinjer kan anvendes til funktionelle analyser herunder immunoblotting, immunofluorescens og patch klemme. Denne sygdom i parabol fremgangsmåde omgår vanskeligheden ved at opnå indfødte ÅV menneskeceller.

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Genetik spørgsmålet 138 humane pluripotente stamceller hPSC retinale pigment epitel hPSC-RPE retinal degenerative sygdomme sygdom i parabol immunoblotting immunofluorescens patch klemme BESTROPHIN1 BEST1 Ca2 +- aktiverede Cl- kanal
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