Generation of an induced pluripotent stem cell line HHUUKDi013-A (ISRM-AATD-iPSC-3) from a pediatric patient of Alpha-I Antitrypsin Deficiency (AATD)

Renal progenitor cells were isolated from urine from a male, pediatric AATD-patient, harboring the homologous Pi*ZZ genotype. SIX2-positive urine derived renal progenitor cells (UdRPCs) were reprogrammed to iPSCs by integration-free nucleofection of episomal-based plasmids expressing OCT4, KLF4, c-MYC, SOX2, NANOG and LIN28. ISRM-AATD-iPSC-3 show pluripotent gene and protein expression, are capable of forming embryoid bodies and carry the parental Pi*ZZ genotype. Global transcriptome analyses revealed a correlation coefficient 0.9091 between the human embryonic stem cell line H9 and ISRM-AATD-iPSC-3.