B6.Cg-Tg(CHMP2B*)153Aisa/AisaH

Status	Available to order
EMMA ID	EM:12513
Citation information	RRID:IMSR_EM:12513 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information.
International strain name	B6.Cg-Tg(CHMP2B*)153Aisa/AisaH
Alternative name	C57BL/6J-Tg(CHMP2B-Intron5)153Aisa
Strain type	Transgenic Strains
Allele/Transgene symbol	Tg(CHMP2B*)153Aisa
Gene/Transgene symbol	Tg(CHMP2B*)153Aisa

Information from provider

Provider	Adrian Isaacs
Provider affiliation	UCL
Genetic information	Human CHMP2B-intron5 cDNA transgene was inserted by random integration into the mouse genome
Phenotypic information	Homozygous: These mice develop pathologies in adult brain, including neuronal lysosomal storage pathology, astrogliosis and microgliosis (Ghazi-Noori et al., Brain. 2012, 135:819-32; Clayton et al., Acta Neuropathol. 2015, 130(4):511-23; Clayton et al Hum Mol Genet. 2017, 26(5):873-887). They also develop late-onset deficits in motor function and social behavior at 18 months of age (Clayton et al., Hum Mol Genet. 2017. 26(5):873-887). Heterozygous: No observable phenotype in hemizygous mice.
Breeding history	Mice were backcrossed to C57BL/6J for 10 generations followed by more than 20 generations of intercrossing of homozygotes.
References	Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice.;Ghazi-Noori Shabnam, Froud Kristina E, Mizielinska Sarah, Powell Caroline, Smidak Michelle, Fernandez de Marco Mar, O'Malley Catherine, Farmer Michael, Parkinson Nick, Fisher Elizabeth M C, Asante Emmanuel A, Brandner Sebastian, Collinge John, Isaacs Adrian M, ;2012;Brain : a journal of neurology;135;819-32; 22366797 Early microgliosis precedes neuronal loss and behavioural impairment in mice with a frontotemporal dementia-causing CHMP2B mutation.;Clayton Emma L, Mancuso Renzo, Nielsen Troels Tolstrup, Mizielinska Sarah, Holmes Holly, Powell Nicholas, Norona Frances, Larsen Jytte Overgaard, Milioto Carmelo, Wilson Katherine M, Lythgoe Mark F, Ourselin Sebastian, Nielsen Jörgen E, Johannsen Peter, Holm Ida, Collinge John, null null, Oliver Peter L, Gomez-Nicola Diego, Isaacs Adrian M, ;2017;Human molecular genetics;26;873-887; 28093491 Frontotemporal dementia caused by CHMP2B mutation is characterised by neuronal lysosomal storage pathology.;Clayton Emma L, Mizielinska Sarah, Edgar James R, Nielsen Troels Tolstrup, Marshall Sarah, Norona Frances E, Robbins Miranda, Damirji Hana, Holm Ida E, Johannsen Peter, Nielsen Jørgen E, Asante Emmanuel A, Collinge John, null null, Isaacs Adrian M, ;2015;Acta neuropathologica;130;511-23; 26358247
Homozygous fertile	yes
Homozygous viable	yes
Homozygous matings required	no
Immunocompromised	no

Information from EMMA

Archiving centre	Mary Lyon Centre at MRC Harwell, Oxford, United Kingdom

Literature references

Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice.;Ghazi-Noori Shabnam, Froud Kristina E, Mizielinska Sarah, Powell Caroline, Smidak Michelle, Fernandez de Marco Mar, O'Malley Catherine, Farmer Michael, Parkinson Nick, Fisher Elizabeth M C, Asante Emmanuel A, Brandner Sebastian, Collinge John, Isaacs Adrian M, ;2012;Brain : a journal of neurology;135;819-32; 22366797
Early microgliosis precedes neuronal loss and behavioural impairment in mice with a frontotemporal dementia-causing CHMP2B mutation.;Clayton Emma L, Mancuso Renzo, Nielsen Troels Tolstrup, Mizielinska Sarah, Holmes Holly, Powell Nicholas, Norona Frances, Larsen Jytte Overgaard, Milioto Carmelo, Wilson Katherine M, Lythgoe Mark F, Ourselin Sebastian, Nielsen Jörgen E, Johannsen Peter, Holm Ida, Collinge John, null null, Oliver Peter L, Gomez-Nicola Diego, Isaacs Adrian M, ;2017;Human molecular genetics;26;873-887; 28093491
Frontotemporal dementia caused by CHMP2B mutation is characterised by neuronal lysosomal storage pathology.;Clayton Emma L, Mizielinska Sarah, Edgar James R, Nielsen Troels Tolstrup, Marshall Sarah, Norona Frances E, Robbins Miranda, Damirji Hana, Holm Ida E, Johannsen Peter, Nielsen Jørgen E, Asante Emmanuel A, Collinge John, null null, Isaacs Adrian M, ;2015;Acta neuropathologica;130;511-23; 26358247

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