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ABCA7 encodes an ATP-binding cassette transporter, and its loss-of-function variants are associated with Alzheimer’s disease. To investigate the role of ABCA7 deficiency in the pathogenesis of Alzheimer’s disease, we generated a homozygous ABCA7-knockout induced pluripotent stem cell (iPSC) line using CRISPR/Cas9-mediated gene editing. This ABCA7-deficient iPSC line maintains a normal karyotype, expression of pluripotency markers, and trilineage differentiation capacity.