Characterisation of PTCD1 and CRYAB variants in rare and mitochondrial disease pathogenesis using gene editing

Mitochondrial diseases are highly complex, debilitating conditions impacting 1 in every 6,000 to 8,000 live births. Investigation of patient variants by generating cellular models is vital to a timely and accurate diagnosis. This thesis demonstrates the first instance of combining prime editing syst...

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Main Author: Mantegna, Jessica Lee
Format: Thesis
Published: Curtin University 2024
Online Access:http://hdl.handle.net/20.500.11937/96637
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author Mantegna, Jessica Lee
author_facet Mantegna, Jessica Lee
author_sort Mantegna, Jessica Lee
building Curtin Institutional Repository
collection Online Access
description Mitochondrial diseases are highly complex, debilitating conditions impacting 1 in every 6,000 to 8,000 live births. Investigation of patient variants by generating cellular models is vital to a timely and accurate diagnosis. This thesis demonstrates the first instance of combining prime editing systems with engineered pegRNAs into one plasmid for delivery into mammalian cells, identified the first disease-causing variant in PTCD1, and characterised three variants in CRYAB to investigate contribution to mitochondrial disease and dysfunction.
first_indexed 2025-11-14T11:47:08Z
format Thesis
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institution Curtin University Malaysia
institution_category Local University
last_indexed 2025-11-14T11:47:08Z
publishDate 2024
publisher Curtin University
recordtype eprints
repository_type Digital Repository
spelling curtin-20.500.11937-966372024-12-20T07:16:18Z Characterisation of PTCD1 and CRYAB variants in rare and mitochondrial disease pathogenesis using gene editing Mantegna, Jessica Lee Mitochondrial diseases are highly complex, debilitating conditions impacting 1 in every 6,000 to 8,000 live births. Investigation of patient variants by generating cellular models is vital to a timely and accurate diagnosis. This thesis demonstrates the first instance of combining prime editing systems with engineered pegRNAs into one plasmid for delivery into mammalian cells, identified the first disease-causing variant in PTCD1, and characterised three variants in CRYAB to investigate contribution to mitochondrial disease and dysfunction. 2024 Thesis http://hdl.handle.net/20.500.11937/96637 Curtin University restricted
spellingShingle Mantegna, Jessica Lee
Characterisation of PTCD1 and CRYAB variants in rare and mitochondrial disease pathogenesis using gene editing
title Characterisation of PTCD1 and CRYAB variants in rare and mitochondrial disease pathogenesis using gene editing
title_full Characterisation of PTCD1 and CRYAB variants in rare and mitochondrial disease pathogenesis using gene editing
title_fullStr Characterisation of PTCD1 and CRYAB variants in rare and mitochondrial disease pathogenesis using gene editing
title_full_unstemmed Characterisation of PTCD1 and CRYAB variants in rare and mitochondrial disease pathogenesis using gene editing
title_short Characterisation of PTCD1 and CRYAB variants in rare and mitochondrial disease pathogenesis using gene editing
title_sort characterisation of ptcd1 and cryab variants in rare and mitochondrial disease pathogenesis using gene editing
url http://hdl.handle.net/20.500.11937/96637