UAMi004-A

Registration Summary:

A P E C

PCCB10-FiPS4F1

The cell line is not submitted yet.

(only basic data is shown)

General

iPSC Line
hPSCreg name	UAMi004-A
Cite as:	UAMi004-A (RRID:CVCL_WT14)
Alternative name(s)	PCCB10-FiPS4F1
iPSC line type	Human induced pluripotent stem cell (hiPSC)
Similar iPSC lines	No similar lines found.
Last update	19th May 2021
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Provider
Generator	Universidad Autonoma de Madrid (UAM)
External iPSC Databases
Cellosaurus	CVCL_WT14
Wikidata	Q98133803
General iPSC Information
Publications	López-Márquez A et al. Generation and characterization of a human iPSC line (UAMi004-A) from a patient with propionic acidemia due to defects in the PCCB gene. Stem cell research. 2019 Jul;38:101469. López-Márquez A et al. Corrigendum to "Generation and characterization of a human iPSC line (UAMi004-A) from a patient with propionic acidemia due to defects in the PCCB gene" [Stem Cell Research, Volume 38, July 2019, 101469]. Stem cell research. 2019 Aug;39:101513. Collado MS et al. Biochemical and anaplerotic applications of in vitro models of propionic acidemia and methylmalonic acidemia using patient-derived primary hepatocytes. Molecular genetics and metabolism. 2020 Jul;130(3):183-196. Fulgencio-Covián A et al. Generation of a gene-corrected human isogenic line (UAMi006-A) from propionic acidemia patient iPSC with an homozygous mutation in the PCCB gene using CRISPR/Cas9 technology. Stem cell research. 2020 Dec;49:102055. McKnight CL et al. Modelling Mitochondrial Disease in Human Pluripotent Stem Cells: What Have We Learned?. International journal of molecular sciences. 2021 Jul 20;22(14). Álvarez M et al. Dysregulated Cell Homeostasis and miRNAs in Human iPSC-Derived Cardiomyocytes from a Propionic Acidemia Patient with Cardiomyopathy. International journal of molecular sciences. 2023 Jan 22;24(3). García-Tenorio EM et al. Novel CRISPR-Cas9 iPSC knockouts for PCCA and PCCB genes: advancing propionic acidemia research. Human cell. 2025 Mar 5;38(3):64. Tao Tianqi et al. Generation of an isogenic human induced pluripotent stem cell line with a mutant propionyl-CoA carboxylase α subunit. Orphanet Journal of Rare Diseases. 2026-01-23.
Subclones	UAMi004-A-1

iPSC Derivation

General