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JoVE Journal Genetics
Differensiering, vedlikehold og analyse av menneskelig netthinnens Pigment epitel celler
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Differensiering, vedlikehold og analyse av menneskelig netthinnens Pigment epitel celler: en sykdom-i-en-fat modell for BEST1 mutasjoner

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 presenterer vi en protokoll for å skille netthinnens pigment epitel (RPE) celler fra menneskelige pluripotent stamceller bærer pasient-avledede mutasjoner. De muterte cellelinjer kan brukes for funksjonell analyser inkludert immunoblotting, immunofluorescence og patch klemme. Denne sykdommen-i-en-fat omgår vanskelighetene med å skaffe innfødt menneskelige RPE celler.

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Genetikk problemet 138 menneskelige pluripotent stamceller hPSC netthinnens pigment epitel hPSC-RPE netthinnens degenerative sykdommer sykdom-i-en-fat immunoblotting immunofluorescence patch klemme BESTROPHIN1 BEST1 Ca2 +- aktivert Cl- kanal
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