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HIHCNi002-A
Registration Summary
:
A
P
E
C
iPSC-SCA3
The cell line is
not submitted
yet.
(only basic data is shown)
General
Cell Line
hPSCreg name
HIHCNi002-A
Cite as:
When citing this cell line, please use the hPSCreg name (see
Naming Tool
) and the corresponding Research Resource ID (RRID).
HIHCNi002-A (RRID:CVCL_VL41)
Alternative name(s)
iPSC-SCA3
Cell line type
Human induced pluripotent stem cell (hiPSC)
Similar lines
No similar lines found.
Last update
14th April 2018
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Provider
Generator
Hertie Institute for Clinical Brain Research - Clinical Neurogenetics (HIHCN)
External Databases
Cellosaurus
CVCL_VL41
Wikidata
Q94208294
General Information
Publications
Harmuth T et al. Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3. Frontiers in molecular neuroscience. 2018;11:368.
Hayer SN et al. Generation of an induced pluripotent stem cell line from a patient with spinocerebellar ataxia type 3 (SCA3): HIHCNi002-A. Stem cell research. 2018 Jul;30:171-174.
Harmuth T et al. Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3. Front Mol Neurosci. 2018-10-10.
Moore LR et al. Antisense oligonucleotide therapy rescues aggresome formation in a novel spinocerebellar ataxia type 3 human embryonic stem cell line. Stem cell research. 2019 Aug;39:101504.
Wang AYL et al. Episomal Induced Pluripotent Stem Cells: Functional and Potential Therapeutic Applications. Cell transplantation. 2019 Dec;28(1_suppl):112S-131S.
He L et al. Human stem cell models of polyglutamine diseases: Sources for disease models and cell therapy. Experimental neurology. 2021 Mar;337:113573.
Gonsior K et al. PolyQ-expanded ataxin-3 protein levels in peripheral blood mononuclear cells correlate with clinical parameters in SCA3: a pilot study. Journal of neurology. 2021 Apr;268(4):1304-1315.
Pereira Sena P et al. Pathophysiological interplay between O-GlcNAc transferase and the Machado-Joseph disease protein ataxin-3. Proceedings of the National Academy of Sciences of the United States of America. 2021 Nov 23;118(47).
Hommersom MP et al. Human Induced Pluripotent Stem Cell-Based Modelling of Spinocerebellar Ataxias. Stem cell reviews and reports. 2022 Feb;18(2):441-456.
Hauser S et al. Allele-specific targeting of mutant ataxin-3 by antisense oligonucleotides in SCA3-iPSC-derived neurons. Molecular therapy. Nucleic acids. 2022 Mar 8;27:99-108.
Abeditashi M et al. KPNB1 modulates the Machado-Joseph disease protein ataxin-3 through activation of the mitochondrial protease CLPP. Cellular and molecular life sciences : CMLS. 2022 Jul 6;79(8):401.
Coelho P et al. Mitochondrial function and dynamics in neural stem cells and neurogenesis: Implications for neurodegenerative diseases. Ageing research reviews. 2022 Sep;80:101667.
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