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@ARTICLE{Lansing:163482,
author = {Lansing, Felix and Mukhametzyanova, Liliya and
Rojo-Romanos, Teresa and Iwasawa, Kentaro and Kimura, Masaki
and Paszkowski-Rogacz, Maciej and Karpinski, Janet and
Grass, Tobias and Sonntag, Jan and Schneider, Paul Martin
and Günes, Ceren and Hoersten, Jenna and Schmitt, Lukas
Theo and Rodriguez-Muela, Natalia and Knöfler, Ralf and
Takebe, Takanori and Buchholz, Frank},
title = {{C}orrection of a {F}actor {VIII} genomic inversion with
designer-recombinases.},
journal = {Nature Communications},
volume = {13},
number = {1},
issn = {2041-1723},
address = {[London]},
publisher = {Nature Publishing Group UK},
reportid = {DZNE-2022-00242},
pages = {422},
year = {2022},
abstract = {Despite advances in nuclease-based genome editing
technologies, correcting human disease-causing genomic
inversions remains a challenge. Here, we describe the
potential use of a recombinase-based system to correct the
140 kb inversion of the F8 gene frequently found in patients
diagnosed with severe Hemophilia A. Employing
substrate-linked directed molecular evolution, we develop a
coupled heterodimeric recombinase system (RecF8) achieving
$30\%$ inversion of the target sequence in human tissue
culture cells. Transient RecF8 treatment of endothelial
cells, differentiated from patient-derived induced
pluripotent stem cells (iPSCs) of a hemophilic donor,
results in $12\%$ correction of the inversion and restores
Factor VIII mRNA expression. In this work, we present
designer-recombinases as an efficient and specific means
towards treatment of monogenic diseases caused by large gene
inversions.},
keywords = {Amino Acid Sequence / Base Sequence / Cell Differentiation
/ Chromosome Inversion: genetics / Clone Cells / Directed
Molecular Evolution / Endothelial Cells: cytology /
Endothelial Cells: metabolism / Exons: genetics / Factor
VIII: genetics / HEK293 Cells / HeLa Cells / Humans /
Induced Pluripotent Stem Cells: metabolism / Inverted Repeat
Sequences: genetics / Recombinases: metabolism /
Recombination, Genetic: genetics / Substrate Specificity /
Whole Genome Sequencing / Recombinases (NLM Chemicals) /
Factor VIII (NLM Chemicals)},
cin = {AG Rodriguez-Muela},
ddc = {500},
cid = {I:(DE-2719)1713001},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:35058465},
pmc = {pmc:PMC8776779},
doi = {10.1038/s41467-022-28080-7},
url = {https://pub.dzne.de/record/163482},
}