% IMPORTANT: The following is UTF-8 encoded. This means that in the presence % of non-ASCII characters, it will not work with BibTeX 0.99 or older. % Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or % “biber”. @ARTICLE{Gttert:276819, author = {Göttert, Ria and Vallone, Valeria Fernandez and Stachelscheid, Harald and Metzger, Jakob Johannes and Caedo, Cassandra Carao and Buthut, Maria and Prüss, Harald and Endres, Matthias and Schilling, Simone and Gertz, Karen}, title = {{G}eneration of a human induced pluripotent stem cell line ({BIH}i292-{A}) from {PBMC}s of a female patient diagnosed with {N}asu-{H}akola disease ({NHD})/polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy ({PLOSL}) carrying a novel heterozygous mutation in the {TREM}2 gene.}, journal = {Stem cell research}, volume = {83}, issn = {1873-5061}, address = {Amsterdam [u.a.]}, publisher = {Elsevier}, reportid = {DZNE-2025-00332}, pages = {103660}, year = {2025}, abstract = {NHD/PLOSL is an orphan disease characterized by progressive presenile dementia associated with recurrent fractures due to polycystic bone lesions. In this study, we generated the human induced pluripotent stem cell (hiPSC) line BIHi292-A from a 30-year-old women diagnosed with NHD/PLOSL, carrying two compound heterozygous frameshift mutations [c.313del (p.Ala105fs) and c.199del (p.His67fs)] in the TREM2 (triggering receptor expressed on myeloid cells 2) gene. BIHi292-A hiPSCs are karyotypically normal, express typical markers for the undifferentiated state and have pluripotent differentiation potential. BIHi292-A cells will provide a valuable tool for investigating pathogenic mechanisms of NHD/PLOSL and TREM2-related research questions.}, keywords = {Humans / Female / Induced Pluripotent Stem Cells: metabolism / Membrane Glycoproteins: genetics / Membrane Glycoproteins: metabolism / Receptors, Immunologic: genetics / Receptors, Immunologic: metabolism / Subacute Sclerosing Panencephalitis: genetics / Subacute Sclerosing Panencephalitis: pathology / Adult / Heterozygote / Osteochondrodysplasias: genetics / Osteochondrodysplasias: pathology / Mutation / Cell Line / Lipodystrophy: genetics / Lipodystrophy: pathology / Cell Differentiation / TREM2 protein, human (NLM Chemicals) / Membrane Glycoproteins (NLM Chemicals) / Receptors, Immunologic (NLM Chemicals)}, cin = {AG Prüß / AG Endres}, ddc = {570}, cid = {I:(DE-2719)1810003 / I:(DE-2719)1811005}, pnm = {353 - Clinical and Health Care Research (POF4-353)}, pid = {G:(DE-HGF)POF4-353}, typ = {PUB:(DE-HGF)16}, pubmed = {pmid:39879812}, doi = {10.1016/j.scr.2025.103660}, url = {https://pub.dzne.de/record/276819}, }