BIONi010-C-A

Registration Summary:

A P E C

BIONi010-C-41 GRIA3 p.A654V

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General

Cell Line
hPSCreg name	BIONi010-C-A
Cite as:	BIONi010-C-A
Alternative name(s)	BIONi010-C-41 GRIA3 p.A654V
Cell line type	Human induced pluripotent stem cell (hiPSC)
Similar lines	No similar lines found.
Last update	29th April 2026
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Provider
Generator	Bioneer (BION)
General Information
Publications	Xiang X et al. The Trem2 R47H Alzheimer's risk variant impairs splicing and reduces Trem2 mRNA and protein in mice but not in humans. Molecular neurodegeneration. 2018 Sep 6;13(1):49. Hasselmann J et al. Development of a Chimeric Model to Study and Manipulate Human Microglia In Vivo. Neuron. 2019 Sep 25;103(6):1016-1033.e10. Reich M et al. Alzheimer's Risk Gene TREM2 Determines Functional Properties of New Type of Human iPSC-Derived Microglia. Frontiers in immunology. 2020;11:617860. Piers TM et al. A locked immunometabolic switch underlies TREM2 R47H loss of function in human iPSC-derived microglia. FASEB journal : official publication of the Federation of American Societies for Experimental Biology. 2020 Feb;34(2):2436-2450. Freude KK et al. Enrichment of retinal ganglion and Müller glia progenitors from retinal organoids derived from human induced pluripotent stem cells - possibilities and current limitations. World journal of stem cells. 2020 Oct 26;12(10):1171-1183. Lauschke K et al. A novel human pluripotent stem cell-based assay to predict developmental toxicity. Archives of toxicology. 2020 Nov;94(11):3831-3846. Hall-Roberts H et al. TREM2 Alzheimer's variant R47H causes similar transcriptional dysregulation to knockout, yet only subtle functional phenotypes in human iPSC-derived macrophages. Alzheimer's research & therapy. 2020 Nov 16;12(1):151. Aires V et al. CD22 Blockage Restores Age-Related Impairments of Microglia Surveillance Capacity. Frontiers in immunology. 2021;12:684430. Mallach A et al. The influence of the R47H triggering receptor expressed on myeloid cells 2 variant on microglial exosome profiles. Brain communications. 2021;3(2):fcab009. Vajhøj C et al. Establishment of a human induced pluripotent stem cell neuronal model for identification of modulators of A53T α-synuclein levels and aggregation. PloS one. 2021;16(12):e0261536. Goldeman C et al. Human induced pluripotent stem cells (BIONi010-C) generate tight cell monolayers with blood-brain barrier traits and functional expression of large neutral amino acid transporter 1 (SLC7A5). European journal of pharmaceutical sciences : official journal of the European Federation for Pharmaceutical Sciences. 2021 Jan 1;156:105577. Magusali Naciye et al. Genetic variability associated with OAS1 expression in myeloid cells increases the risk of Alzheimer’s disease and severe COVID-19 outcomes. . 2021-03-18. Schmid B et al. Generation of two gene edited iPSC-lines carrying a DOX-inducible NGN2 expression cassette with and without GFP in the AAVS1 locus. Stem cell research. 2021 Apr;52:102240. Lauschke K et al. Creating a human-induced pluripotent stem cell-based NKX2.5 reporter gene assay for developmental toxicity testing. Archives of toxicology. 2021 May;95(5):1659-1670. Vigh JP et al. Transendothelial Electrical Resistance Measurement across the Blood-Brain Barrier: A Critical Review of Methods. Micromachines. 2021 Jun 11;12(6). Cosker K et al. Microglial signalling pathway deficits associated with the patient derived R47H TREM2 variants linked to AD indicate inability to activate inflammasome. Scientific reports. 2021 Jun 25;11(1):13316. Wißfeld J et al. Reporter cell assay for human CD33 validated by specific antibodies and human iPSC-derived microglia. Scientific reports. 2021 Jun 29;11(1):13462. Nazish I et al. Abrogation of LRRK2 dependent Rab10 phosphorylation with TLR4 activation and alterations in evoked cytokine release in immune cells. Neurochemistry international. 2021 Jul;147:105070. Shih PY et al. Development of a fully human assay combining NGN2-inducible neurons co-cultured with iPSC-derived astrocytes amenable for electrophysiological studies. Stem cell research. 2021 Jul;54:102386. Obst J et al. PLCγ2 regulates TREM2 signalling and integrin-mediated adhesion and migration of human iPSC-derived macrophages. Scientific reports. 2021 Oct 6;11(1):19842. Mallach A et al. Differential Stimulation of Pluripotent Stem Cell-Derived Human Microglia Leads to Exosomal Proteomic Changes Affecting Neurons. Cells. 2021 Oct 24;10(11). (unknown author). Abstracts from the Virtual Symposium "Signal Transduction at the Blood-Brain Barriers". Fluids and barriers of the CNS. 2021 Dec 17;18(Suppl 1):58. Fagerlund I et al. Microglia-like Cells Promote Neuronal Functions in Cerebral Organoids. Cells. 2021 Dec 30;11(1). Magusali N et al. A genetic link between risk for Alzheimer's disease and severe COVID-19 outcomes via the OAS1 gene. Brain : a journal of neurology. 2021 Dec 31;144(12):3727-3741. Giovenale AMG et al. Deepening the understanding of CNVs on chromosome 15q11-13 by using hiPSCs: An overview. Frontiers in cell and developmental biology. 2022;10:1107881. de Leeuw SM et al. APOE2, E3, and E4 differentially modulate cellular homeostasis, cholesterol metabolism, and inflammatory response in isogenic iPSC-derived astrocytes. Stem cell reports. 2022 Jan 11;17(1):110-126. Manos JD et al. Uncovering specificity of endogenous TAU aggregation in a human iPSC-neuron TAU seeding model. iScience. 2022 Jan 21;25(1):103658. Bull D et al. Generation of an hiPSC-Derived Co-Culture System to Assess the Effects of Neuroinflammation on Blood-Brain Barrier Integrity. Cells. 2022 Jan 26;11(3). Sampathkumar NK et al. RNA-Seq is not required to determine stable reference genes for qPCR normalization. PLoS computational biology. 2022 Feb;18(2):e1009868. Morera Cristina et al. CRISPR-mediated correction of skeletal muscle Ca ²⁺ handling in a novel DMD patient-derived pluripotent stem cell model. . 2022-02-18. Solomon Shiden et al. Heterozygous expression of the Alzheimer’s disease protective PLCγ2 P522R variant enhances Aβ clearance while preserving synapses. . 2022-04-20. Wei Jingzhang et al. Glucocorticoid receptor activation during human microglial differentiation leads to genomic instability and senescence. . 2022-05-23. Jäntti H et al. Microglial amyloid beta clearance is driven by PIEZO1 channels. Journal of neuroinflammation. 2022 Jun 15;19(1):147. Solomon S et al. Heterozygous expression of the Alzheimer's disease-protective PLCγ2 P522R variant enhances Aβ clearance while preserving synapses. Cellular and molecular life sciences : CMLS. 2022 Jul 27;79(8):453. Luconi M et al. Human-Based New Approach Methodologies in Developmental Toxicity Testing: A Step Ahead from the State of the Art with a Feto-Placental Organ-on-Chip Platform. International journal of environmental research and public health. 2022 Nov 28;19(23). Boudesco C et al. Novel potent liposome agonists of triggering receptor expressed on myeloid cells 2 phenocopy antibody treatment in cells. Glia. 2022 Dec;70(12):2290-2308. Pantazis CB et al. A reference human induced pluripotent stem cell line for large-scale collaborative studies. Cell stem cell. 2022 Dec 1;29(12):1685-1702.e22. Stolzenburg LR et al. Functional characterization of a single nucleotide polymorphism associated with Alzheimer's disease in a hiPSC-based neuron model. PloS one. 2023;18(9):e0291029. Mathews M et al. Reenacting Neuroectodermal Exposure of Hematopoietic Progenitors Enables Scalable Production of Cryopreservable iPSC-Derived Human Microglia. Stem cell reviews and reports. 2023 Feb;19(2):455-474. Somogyi A et al. The synthetic TRPML1 agonist ML-SA1 rescues Alzheimer-related alterations of the endosomal-autophagic-lysosomal system. Journal of cell science. 2023 Mar 15;136(6). Lee H et al. In vitro characterization on the role of APOE polymorphism in human hippocampal neurogenesis. Hippocampus. 2023 Apr;33(4):322-346. Popescu AS et al. Alzheimer's disease-associated R47H TREM2 increases, but wild-type TREM2 decreases, microglial phagocytosis of synaptosomes and neuronal loss. Glia. 2023 Apr;71(4):974-990. Gehrlein A et al. Targeting neuronal lysosomal dysfunction caused by β-glucocerebrosidase deficiency with an enzyme-based brain shuttle construct. Nature communications. 2023 Apr 12;14(1):2057. Meiser I et al. Application-Oriented Bulk Cryopreservation of Human iPSCs in Cryo Bags Followed by Direct Inoculation in Scalable Suspension Bioreactors for Expansion and Neural Differentiation. Cells. 2023 Jul 22;12(14). Sinner P et al. Microglial expression of CD83 governs cellular activation and restrains neuroinflammation in experimental autoimmune encephalomyelitis. Nature communications. 2023 Aug 1;14(1):4601. Faruqui N et al. Extracellular matrix type 0: From ancient collagen lineage to a versatile product pipeline - JellaGel™. Materials today. Bio. 2023 Oct;22:100786. Lim CM et al. Multiomic prediction of therapeutic targets for human diseases associated with protein phase separation. Proceedings of the National Academy of Sciences of the United States of America. 2023 Oct 3;120(40):e2300215120. Sarieva K et al. Pluripotent stem cell-derived neural progenitor cells can be used to model effects of IL-6 on human neurodevelopment. Disease models & mechanisms. 2023 Nov 1;16(11). Müller M et al. Alveolar epithelial-like cell differentiation in a dynamic bioreactor: a promising 3D-approach for the high-throughput generation of lung cell types from human induced pluripotent stem cells. In vitro models. 2023 Dec;2(6):249-262. Sarieva K et al. Human brain organoid model of maternal immune activation identifies radial glia cells as selectively vulnerable. Molecular psychiatry. 2023 Dec;28(12):5077-5089. Ozgür B et al. Characterization of an iPSC-based barrier model for blood-brain barrier investigations using the SBAD0201 stem cell line. Fluids and barriers of the CNS. 2023 Dec 19;20(1):96. Jäntti H et al. Particulate matter from car exhaust alters function of human iPSC-derived microglia. Particle and fibre toxicology. 2024 Feb 15;21(1):6. Gallego Villarejo L et al. Optical Genome Mapping Reveals Genomic Alterations upon Gene Editing in hiPSCs: Implications for Neural Tissue Differentiation and Brain Organoid Research. Cells. 2024 Mar 14;13(6). Kaurani L et al. Regulation of Zbp1 by miR-99b-5p in microglia controls the development of schizophrenia-like symptoms in mice. The EMBO journal. 2024 Apr;43(8):1420-1444. Nielsen AKR et al. Generation of an iPSC-line (BIONi010C-48) with restored P-glycoprotein functionality following transfection with the human MDR1 gene in the AAVS1 locus. Stem cell research. 2024 Apr;76:103348. Treschow AF et al. Investigating the applicability domain of the hiPSC-based PluriLum assay: an embryotoxicity assessment of chemicals and drugs. Archives of toxicology. 2024 Apr;98(4):1209-1224. Vandermeulen L et al. Regulation of human microglial gene expression and function via RNAase-H active antisense oligonucleotides in vivo in Alzheimer's disease. Molecular neurodegeneration. 2024 Apr 24;19(1):37. Mancuso R et al. Xenografted human microglia display diverse transcriptomic states in response to Alzheimer's disease-related amyloid-β pathology. Nature neuroscience. 2024 May;27(5):886-900. Cosker Katharina et al. Fibrinogen signalling in human iPSC-derived microglia. . 2024-05-06. Öttl M et al. Reduced synaptic depression in human neurons carrying homozygous disease-causing STXBP1 variant L446F. Human molecular genetics. 2024 May 18;33(11):991-1000. Budny V et al. APOE4 Increases Energy Metabolism in APOE-Isogenic iPSC-Derived Neurons. Cells. 2024 Jul 17;13(14). Duggan MR et al. Proteome-wide analysis identifies plasma immune regulators of amyloid-beta progression. Brain, behavior, and immunity. 2024 Aug;120:604-619. Díaz Alicia González et al. A human neuronal model of sporadic Alzheimer’s disease induced by FBXO2 downregulation shows Aβ aggregation, tau hyperphosphorylation and functional network impairment. . 2024-09-03. Lee SE et al. Hippocampal tau-induced GRIN3A deficiency in Alzheimer's disease. FEBS open bio. 2024 Dec;14(12):2059-2071. Treschow AF et al. Standardization and optimization of the hiPSC-based PluriLum assay for detection of embryonic and developmental toxicants. Archives of toxicology. 2024 Dec;98(12):4107-4116. Haustein MD et al. Development and characterization of a scalable calcium imaging assay using human iPSC-derived neurons. Frontiers in cellular neuroscience. 2025;19:1701907. Policarpo R et al. The MIR-NAT MAPT-AS1 does not regulate Tau expression in human neurons. PloS one. 2025;20(1):e0314973. Sébastien M et al. Measurements of neurite extension and nucleokinesis in an iPSC-derived model system following microtubule perturbation. Molecular biology of the cell. 2025 Jan 1;36(1):mr1. Barulin A et al. Axially multifocal metalens for 3D volumetric photoacoustic imaging of neuromelanin in live brain organoid. Science advances. 2025 Jan 17;11(3):eadr0654. Jeong JH et al. Long-term exposure to excessive norepinephrine in the brain induces tau aggregation, neuronal death, and cognitive deficits in early tau transgenic mice. Aging cell. 2025 Mar;24(3):e14420. Meier S et al. An efficient, non-viral arrayed CRISPR screening platform for iPSC-derived myeloid and microglia models. Stem cell reports. 2025 Mar 11;20(3):102420. Vasilopoulou F et al. Amelioration of signaling deficits underlying metabolic shortfall in TREM2(R47H) human iPSC-derived microglia. The FEBS journal. 2025 Apr;292(7):1743-1762. Guo W et al. Emerging Perfluorinated Chemical GenX: Environmental and Biological Fates and Risks. Environment & health (Washington, D.C.). 2025 Apr 18;3(4):338-351. Weidling I et al. hiPSC-neurons recapitulate the subtype-specific cell intrinsic nature of susceptibility to neurodegenerative disease-relevant aggregation. Acta neuropathologica communications. 2025 May 19;13(1):108. Murphy KB et al. The APOE isoforms differentially shape the transcriptomic and epigenomic landscapes of human microglia xenografted into a mouse model of Alzheimer's disease. Nature communications. 2025 May 27;16(1):4883. van Voorst Torben W. et al. One-step induction of human GABAergic neurons promotes presynaptic development & synapse maturation. . 2025-07-04. Habich C et al. BMP, MEK, and WNT inhibition with NGN2 expression for rapid generation of hiPSC-derived neurons amenable to regional patterning. Stem cell reports. 2025 Jul 8;20(7):102539. Yentür Z et al. Human dorsal forebrain organoids show differentiation-state-specific protein secretion. iScience. 2025 Jul 18;28(7):112935. Thiab S et al. Human-Induced Pluripotent Stem Cells (iPSCs) for Disease Modeling and Insulin Target Cell Regeneration in the Treatment of Insulin Resistance: A Review. Cells. 2025 Aug 1;14(15). Flores BN et al. Investigational eIF2B activator DNL343 modulates the integrated stress response in preclinical models of TDP-43 pathology and individuals with ALS in a randomized clinical trial. Nature communications. 2025 Aug 18;16(1):7690. Perez-Alcantara Marta et al. Integrated QTL mapping and CRISPR screening in pooled iPSC-derived microglia reveals genetic drivers of neurodegenerative risk. . 2025-08-19. Jo Y et al. Monitoring the Dynamics of Alzheimer's Disease Biomarkers and the APOE-Tau Axis via Human Cerebral Organoids with Immuno-SERS. Advanced science (Weinheim, Baden-Wurttemberg, Germany). 2025 Sep;12(35):e05660. Vasilopoulou Foteini et al. Impact of iPSC-derived microglial exosomes on neurons: Role of TREM2 and implication in Alzheimer's Disease. . 2025-09-17. Wihan J et al. Alpha synuclein-mediated cytoskeletal dysfunction impairs myelination in human oligodendrocytes. Acta neuropathologica. 2025 Sep 19;150(1):33. Das S et al. The nerve center of organ engineering. Nature communications. 2025 Nov 7;16(1):9834. Wu YC et al. APPswe mutation causes functional deficits in endothelial cells generated by transient ETV2 overexpression in human iPSCs. Fluids and barriers of the CNS. 2025 Nov 21;22(1):118. Abramian A et al. Growth factor supplementation modulates survival, morphology, and network activity of neurogenin-2 induced human neurons. Scientific reports. 2025 Dec 13;16(1):2118. Ameen AO et al. Metabolic Astrocytic Support with Decanoic Acid Enhances Energy Metabolism in Alzheimer's Disease Models. Cells. 2025 Dec 16;14(24). Heiss CN et al. The temporal and stimuli-specific effects of LPS and IFNγ on microglial activation. Frontiers in aging neuroscience. 2026;18:1756410. Becker EBE et al. Generating cerebellar organoids from pluripotent stem cells. Disease models & mechanisms. 2026 Jan 1;19(1). Treschow AF et al. Embryotoxicity and mixture effects of legacy PFAS in a human iPSC-based 3D model. Cell biology and toxicology. 2026 Jan 19;42(1):24. Ajith I et al. Assessing Aβ‐independent effects of Module 42 on immune function in vitro. Alzheimers Dement. 2026-02-01. Sarieva K et al. Comparing the impact of sample multiplexing approaches for single-cell RNA-sequencing on downstream analysis using cerebellar organoids. iScience. 2026 Feb 20;29(2):114780. Habich C et al. Protocol for combining BMP, MEK, WNT inhibition and iNGN2 to enable rapid neuronal differentiation and regional patterning from hiPSCs. STAR protocols. 2026 Mar 20;7(1):104385. Harberts J et al. Poking Pluripotency: Nanoinjection Into Human iPSCs. Advanced materials (Deerfield Beach, Fla.). 2026 Apr;38(20):e21046. Vos N et al. Melanocortin 3 Receptors Do Not Specifically Localize to Primary Cilia in Cultured Human and Rodent Neurons. Cell biochemistry and function. 2026 Apr;44(4):e70216.
Subclone of	BIONi010-C

hIPSC Derivation

General
The source cell information can be found in the parental cell line BIONi010-C.