B6;129S2-Sqstm1^tm1Biat/Biat

Status	Available to order
EMMA ID	EM:10848
Citation information	RRID:IMSR_EM:10848 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;129S2-Sqstm1^tm1Biat/Biat
Alternative name	B6N;129S2-Sqstm-tm1Biat, p62-floxed/floxed or p62-f/f
Strain type	Targeted Mutant Strains : Conditional mutation
Allele/Transgene symbol	Sqstm1^tm1Biat
Gene/Transgene symbol	Sqstm1

Information from provider

Provider	Thomas Ruelicke
Provider affiliation	Department 1 (Biomedical Sciences), University of Veterinary Medicine, Vienna
Genetic information	The B6N;129S2-Sqstm1^tm1Biat mice were generated using the Cre/loxP strategy. 5-prime fragment of p62/Sqstm1 gene (NC_000077) containing exons 1–5 was PCR-amplified and inserted via SacI into the pBluescript vector (kindly provided by C. Birchmeier; Max-Delbrueck-Centre for Molecular Medicine, Berlin, Germany) upstream of the loxP-neomycin-loxP cassette. An additional loxP site was introduced before exon 5 using the QuickChange Site-Directed Mutagenesis protocol (Stratagene, La Jolla, CA). To complete the construct, the PCR-amplified 3-prime fragment of p62/Sqstm1 gene was ligated via SalI into the vector and the BamHI-SalI cleaved product was introduced into a wild-type ES (embryonic stem) cell line with a 129S2/SvPas background by electroporation. Neomycin resistant colonies were screened for homologous recombination by long-range PCR and the positive clones with a single integration event were microinjected into C57BL/6 blastocysts. Chimeric males were mated with C57BL/6-Tg(Meu-Cre40) animals to obtain mice which lacked the neo-selection cassette but contained floxed exons 1 to 4 of the Sqstm1 gene (Sqstm1^tm1Biat, p62floxed/floxed or p62f/f). [1]. Postic C, Shiota M, Niswender KD, Jetton TL, Chen Y, Moates JM, et al. Dual Roles for Glucokinase in Glucose Homeostasis as Determined by Liver and Pancreatic beta Cell-specific Gene Knock-outs Using Cre Recombinase. Journal of Biological Chemistry. 1999; 274(1):305–15. PMID: 9867845 [2]. Leneuve P, Colnot S, Hamard G, Francis F, Niwa-Kawakita M, Giovannini M, et al. Cre-mediated germline mosaicism: a new transgenic mouse for the selective removal of residual markers from tri-lox conditional alleles. Nucleic Acids Research. 2003; 31(5):e21. PMID: 12595570
Phenotypic information	Homozygous: no phenotype Heterozygous: no phenotype
References	p62/Sequestosome-1 Is Indispensable for Maturation and Stabilization of Mallory-Denk Bodies.;Lahiri Pooja, Schmidt Volker, Smole Claudia, Kufferath Iris, Denk Helmut, Strnad Pavel, Rülicke Thomas, Fröhlich Leopold F, Zatloukal Kurt, ;2016;PloS one;11;e0161083; 27526095
Homozygous fertile	yes
Homozygous viable	yes
Homozygous matings required	no
Immunocompromised	no

Information from EMMA

Archiving centre	University of Veterinary Medicine, Vienna, Austria
Animals used for archiving	homozygous Other (please specify below) males

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Amyotrophic lateral sclerosis / Orphanet_803
Frontotemporal dementia with motor neuron disease / Orphanet_275872
Behavioral variant of frontotemporal dementia / Orphanet_275864

IMPC phenotypes (gene matching)

increased body length / IMPC
abnormal behavior / IMPC
decreased mean corpuscular hemoglobin / IMPC
decreased circulating glucose level / IMPC
process of degenerative change / IMPC
increased circulating alkaline phosphatase level / IMPC
hypoplasia / IMPC
decreased circulating HDL cholesterol level / IMPC
decreased circulating cholesterol level / IMPC
increased lean body mass / IMPC
decreased circulating LDL cholesterol level / IMPC
decreased mean corpuscular volume / IMPC
decreased hemoglobin content / IMPC

MGI phenotypes (gene matching)

abnormal trabecular bone morphology / MGI
kyphosis / MGI
lordosis / MGI
increased body length / MGI
increased body weight / MGI
abnormal osteoclast physiology / MGI
hyperglycemia / MGI
premature death / MGI
abnormal bone mineralization / MGI
increased susceptibility to age related obesity / MGI
oxidative stress / MGI
premature aging / MGI
abnormal bone structure / MGI
abnormal compact bone morphology / MGI
abnormal behavior / MGI
abnormal osteoclast morphology / MGI
increased osteoclast cell number / MGI
decreased osteoclast cell number / MGI
decreased oxygen consumption / MGI
impaired glucose tolerance / MGI
skeleton phenotype / MGI
decreased circulating glucose level / MGI
decreased mean corpuscular hemoglobin / MGI
increased circulating leptin level / MGI
abnormal mitochondrial physiology / MGI
abnormal bone ossification / MGI
abnormal osteoclast differentiation / MGI
decreased interleukin-6 secretion / MGI
decreased subcutaneous adipose tissue amount / MGI
increased neuron number / MGI
rough coat / MGI
abnormal respiratory electron transport chain / MGI
abnormal aerobic respiration / MGI
increased food intake / MGI

Literature references

p62/Sequestosome-1 Is Indispensable for Maturation and Stabilization of Mallory-Denk Bodies.;Lahiri Pooja, Schmidt Volker, Smole Claudia, Kufferath Iris, Denk Helmut, Strnad Pavel, Rülicke Thomas, Fröhlich Leopold F, Zatloukal Kurt, ;2016;PloS one;11;e0161083; 27526095

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Availabilities

Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740^*
Rederivation of mice from frozen stock, delivery time available upon request . €3880^*

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

^* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

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Practical information

Example health report
(Current health report will be provided later)

Material Transfer Agreement (MTA)
Distribution of this strain is subject to a provider MTA. Both signing of the MTA and submission of the online EMMA Mutant Request Form are required before material can be shipped.

EMMA conditions
Legally binding conditions for the transfer

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B6;129S2-Sqstm1tm1Biat/Biat