B6.Cg-Ikbkg^tm1.1Mpa/Flmg

Status	Available to order
EMMA ID	EM:06968
Citation information	RRID:IMSR_EM:06968 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-Ikbkg^tm1.1Mpa/Flmg
Alternative name	Nemo FL [B6-Ikbkgtm1.1Mpa]
Strain type	Targeted Mutant Strains : Conditional mutation
Allele/Transgene symbol	Ikbkg^tm1.1Mpa
Gene/Transgene symbol	Ikbkg

Information from provider

Provider	Manolis Pasparakis
Provider affiliation	Institut fuer Genetik, Universitaet zu Koeln
Genetic information	This mutant line carries the conditional, floxed Nemo (Ikbkg) mutant allele (MGI symbol: Ikbkg^tm1.1Mpa). Targeted ES cell clones contain the loxP flanked (floxed) Nemo allele and the FRT site-flanked neo-resistance cassette (NemoFN/Y genotype). NEMO-deficient ES cells (NemoD/Y) were generated using cre- and flp-mediated recombination.
Phenotypic information	Disruption of the X-linked gene encoding NF-kappa B essential modulator (NEMO) produces male embryonic lethality, completely blocks NF-kappa B activation by proinflammatory cytokines, and interferes with the generation and/or persistence of lymphocytes. Heterozygous female mice develop patchy skin lesions with massive granulocyte infiltration and hyperproliferation and increased apoptosis of keratinocytes. Diseased animals present severe growth retardation and early mortality. Surviving mice recover almost completely, presumably through clearing the skin of NEMO-deficient keratinocytes.
Breeding history	Bred with C57BL/6J
References	NEMO/IKK gamma-deficient mice model incontinentia pigmenti.;Schmidt-Supprian M, Bloch W, Courtois G, Addicks K, Israël A, Rajewsky K, Pasparakis M, ;2000;Molecular cell;5;981-92; 10911992 Temporally Distinct Functions of the Cytokines IL-12 and IL-23 Drive Chronic Colon Inflammation in Response to Intestinal Barrier Impairment.;Eftychi Christina, Schwarzer Robin, Vlantis Katerina, Wachsmuth Laurens, Basic Marijana, Wagle Prerana, Neurath Markus F, Becker Christoph, Bleich André, Pasparakis Manolis, ;2019;Immunity;51;367-380.e4; 31350179
Homozygous fertile	not known
Homozygous viable	no
Homozygous matings required	no
Immunocompromised	no

Information from EMMA

Archiving centre	B.S.R.C. Alexander Fleming, Vari, Greece
Animals used for archiving	heterozygous C57BL/6 males

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Anhidrotic ectodermal dysplasia-immunodeficiency-osteopetrosis-lymphedema syndrome / Orphanet_69088
X-linked mendelian susceptibility to mycobacterial diseases due to IKBKG deficiency / Orphanet_319612
Hypohidrotic ectodermal dysplasia with immunodeficiency / Orphanet_98813
Incontinentia pigmenti / Orphanet_464

MGI phenotypes (gene matching)

sparse hair / MGI
retarded hair growth / MGI
enlarged spleen / MGI
small spleen / MGI
small thymus / MGI
tremors / MGI
scaly skin / MGI
spontaneous skin ulceration / MGI
thick epidermis / MGI
abnormal epidermis stratum basale morphology / MGI
abnormal epidermis suprabasal layer morphology / MGI
hyperkeratosis / MGI
decreased body weight / MGI
abnormal locomotor behavior / MGI
postnatal growth retardation / MGI
abnormal humoral immune response / MGI
reduced female fertility / MGI
abnormal fertility/fecundity / MGI
no abnormal phenotype detected / MGI
abnormal bone marrow cell morphology/development / MGI
abnormal keratinocyte morphology / MGI
parakeratosis / MGI
erythroderma / MGI
increased hepatocyte apoptosis / MGI
skin inflammation / MGI
decreased B cell number / MGI
increased apoptosis / MGI
absent megakaryocytes / MGI
decreased tumor necrosis factor secretion / MGI
decreased interleukin-12b secretion / MGI
decreased interleukin-6 secretion / MGI
abnormal chemokine secretion / MGI
decreased susceptibility to endotoxin shock / MGI
absent subcutaneous adipose tissue / MGI
abnormal dermal pigmentation / MGI
abnormal epidermal pigmentation / MGI
increased keratinocyte apoptosis / MGI
postnatal lethality, incomplete penetrance / MGI

Literature references

NEMO/IKK gamma-deficient mice model incontinentia pigmenti.;Schmidt-Supprian M, Bloch W, Courtois G, Addicks K, Israël A, Rajewsky K, Pasparakis M, ;2000;Molecular cell;5;981-92; 10911992
Temporally Distinct Functions of the Cytokines IL-12 and IL-23 Drive Chronic Colon Inflammation in Response to Intestinal Barrier Impairment.;Eftychi Christina, Schwarzer Robin, Vlantis Katerina, Wachsmuth Laurens, Basic Marijana, Wagle Prerana, Neurath Markus F, Becker Christoph, Bleich André, Pasparakis Manolis, ;2019;Immunity;51;367-380.e4; 31350179

Information on how we integrate external resources can be found here

Order

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).

More details on pricing and delivery times

Practical information

Genotyping protocol

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

Material Transfer Agreement (MTA)
For this strain no provider MTA is needed. Distribution is based on the EMMA conditions only.

EMMA conditions
Legally binding conditions for the transfer

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B6.Cg-Ikbkgtm1.1Mpa/Flmg