MRL.Cg-Ccr1^tm1Gao Fas^lpr/Cnbc

Status	Available to order
EMMA ID	EM:08422
International strain name	MRL.Cg-Ccr1^tm1Gao Fas^lpr/Cnbc
Alternative name	1L-M
Strain type	Spontaneous
Allele/Transgene symbol	Fas^lpr, Ccr1^tm1Gao
Gene/Transgene symbol	Fas, Ccr1

Information from provider

Provider	Bruno Luckow
Provider affiliation	Arbeitsgruppe Klinische Biochemie, Klinikum der Universität München, Medizinische Klinik und Poliklinik IV
Genetic information	The chemokine receptor Ccr1 has been inactivated by homologous recombination and the resulting Ccr1-deficient mice have been backcrossed to the autoimmune background MRL/lpr, which contains the spontaneous lpr mutation at the Fas locus.
Phenotypic information	Homozygous: Mice homozygous for the lymphoproliferation spontaneous mutation (Fas) show systemic autoimmunity, massive lymphadenopathy associated with proliferation of aberrant T cells, arthritis, and immune complex glomerulonephrosis. Autoimmune disease starts at about 3-4 months of age. The deletion of the chemokine receptor Ccr1 increases severity of the disease. Heterozygous: Heterozygous mice at the lpr locus show a much milder form of the autoimmune disease.
Breeding history	The Ccr1-deficient mice were originally on a C57BL/6 background and the mutant allele has been transferred to the MRL/lpr background by backcrossing. Initially for 7 generations, then after several generations of inbreeding homozygous mice, they have been further backcrossed to N10. Currently they are at N12.
References	None available
Homozygous fertile	yes
Homozygous viable	yes
Homozygous matings required	no
Immunocompromised	not known

Information from EMMA

Archiving centre	CNB-CSIC, Centro Nacional de Biotecnologia, Madrid, Spain
Animals used for archiving	homozygous 0

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Autoimmune lymphoproliferative syndrome / Orphanet_3261

MGI phenotypes (allele matching)

altered response to myocardial infarction / MGI
abnormal leukocyte adhesion / MGI
abnormal cellular extravasation / MGI
decreased mast cell number / MGI
decreased susceptibility to type III hypersensitivity reaction / MGI
abnormal Kupffer cell morphology / MGI
impaired hematopoiesis / MGI
abnormal neutrophil physiology / MGI
granulomatous inflammation / MGI
increased susceptibility to parasitic infection / MGI
immune system phenotype / MGI
increased susceptibility to fungal infection / MGI
abnormal stria vascularis morphology / MGI
increased neutrophil cell number / MGI
cortical renal glomerulopathies / MGI
enlarged lymph nodes / MGI
impaired coordination / MGI
abnormal spatial learning / MGI
decreased IgG level / MGI
conjunctivitis / MGI
vasculitis / MGI
salivary gland inflammation / MGI
premature death / MGI
abnormal kidney physiology / MGI
abnormal lymph node morphology / MGI
increased immunoglobulin level / MGI
abnormal immunoglobulin level / MGI
increased IgG level / MGI
glomerulonephritis / MGI
albuminuria / MGI
increased urine protein level / MGI
dilated renal glomerular capsule / MGI
increased autoantibody level / MGI
abnormal strial intermediate cell morphology / MGI
abnormal conjunctival epithelium morphology / MGI
increased anti-double stranded DNA antibody level / MGI
increased anti-nuclear antigen antibody level / MGI
increased susceptibility to systemic lupus erythematosus / MGI
abnormal renal glomerulus morphology / MGI
abnormal myocardium layer morphology / MGI
vision/eye phenotype / MGI
abnormal enzyme/coenzyme activity / MGI
CNS inflammation / MGI
increased plasma cell number / MGI
podocyte foot process effacement / MGI
decreased marginal zone B cell number / MGI
decreased transitional stage B cell number / MGI
decreased mature B cell number / MGI
decreased immature B cell number / MGI
decreased spleen germinal center number / MGI
renal glomerulus hypertrophy / MGI
expanded mesangial matrix / MGI
mesangial cell hyperplasia / MGI
glomerular crescent / MGI
increased or absent threshold for auditory brainstem response / MGI
abnormal thyroid gland morphology / MGI
enlarged spleen / MGI
enlarged thymus / MGI
skin lesions / MGI
abnormal cardiovascular system physiology / MGI
abnormal immune system physiology / MGI
vascular inflammation / MGI
abnormal gland morphology / MGI
abnormal thymus medulla morphology / MGI
abnormal T cell physiology / MGI
increased IgM level / MGI
increased IgA level / MGI
abnormal type III hypersensitivity reaction / MGI
abnormal immune system organ morphology / MGI
joint swelling / MGI
increased circulating serum albumin level / MGI
thyroid inflammation / MGI
thymus atrophy / MGI
abnormal submandibular gland morphology / MGI
abnormal coronary artery morphology / MGI
abnormal thyroid follicle morphology / MGI
increased anti-single stranded DNA antibody level / MGI
increased anti-erythrocyte antigen antibody level / MGI
abnormal B cell morphology / MGI
increased thymus weight / MGI
abnormal cytotoxic T cell physiology / MGI
abnormal T cell proliferation / MGI
increased susceptibility to autoimmune disorder / MGI
endocrine/exocrine gland phenotype / MGI
abnormal circulating protein level / MGI
abnormal T-helper 2 physiology / MGI
increased circulating total protein level / MGI
abnormal T cell morphology / MGI
lymph node hyperplasia / MGI
enlarged Peyer's patches / MGI
abnormal marginal zone B cell morphology / MGI
increased IgG1 level / MGI
increased IgG2a level / MGI
increased IgG2b level / MGI
decreased interleukin-2 secretion / MGI
abnormal interleukin level / MGI
abnormal B cell receptor editing / MGI
abnormal lymph node cell ratio / MGI
submandibular gland inflammation / MGI
lacrimal gland inflammation / MGI
abnormal glomerular capillary morphology / MGI
renal cast / MGI
increased renal glomerulus basement membrane thickness / MGI
increased kidney cell proliferation / MGI
abnormal glomerular capillary endothelium morphology / MGI
thymus cortex atrophy / MGI
abnormal hepatocyte morphology / MGI
abnormal liver physiology / MGI
demyelination / MGI
focal hepatic necrosis / MGI
brain inflammation / MGI
abnormal kidney morphology / MGI
increased susceptibility to bacterial infection / MGI
increased susceptibility to viral infection / MGI
increased anti-histone antibody level / MGI
increased follicular B cell number / MGI
increased marginal zone B cell number / MGI
increased transitional stage B cell number / MGI
increased immature B cell number / MGI
decreased B cell apoptosis / MGI
abnormal splenic cell ratio / MGI
decreased hepatocyte apoptosis / MGI
increased splenocyte number / MGI
abnormal NK cell physiology / MGI
abnormal renal glomerulus basement membrane morphology / MGI
increased renal glomerulus apoptosis / MGI
increased spleen weight / MGI
increased blood urea nitrogen level / MGI
abnormal airway responsiveness / MGI
decreased eosinophil cell number / MGI
increased apoptosis / MGI
abnormal B cell activation / MGI
mortality/aging / MGI
decreased activated T cell number / MGI
autoimmune response / MGI
decreased B cell number / MGI
increased double-negative T cell number / MGI
decreased CD4-positive, alpha beta T cell number / MGI
decreased CD8-positive, alpha-beta T cell number / MGI
abnormal leukocyte morphology / MGI
impaired leukocyte tethering or rolling / MGI
ruffled hair / MGI
increased body weight / MGI
increased tumor incidence / MGI
decreased susceptibility to autoimmune diabetes / MGI
abnormal skin condition / MGI
epidermal necrosis / MGI
kidney failure / MGI
decreased neuron apoptosis / MGI
decreased apoptosis / MGI
abnormal vagina weight / MGI
lacrimal gland atrophy / MGI
cardiovascular system phenotype / MGI
abnormal stria vascularis vasculature morphology / MGI
increased susceptibility to type III hypersensitivity reaction / MGI
increased sensitivity to induced morbidity/mortality / MGI
decreased macrophage apoptosis / MGI
increased IgG3 level / MGI
kidney inflammation / MGI
increased gamma-delta T cell number / MGI
abnormal immune system morphology / MGI

MGI phenotypes (gene matching)

decreased mast cell number / MGI
altered response to myocardial infarction / MGI
impaired hematopoiesis / MGI
lung inflammation / MGI
decreased airway responsiveness / MGI
decreased susceptibility to viral infection / MGI
decreased susceptibility to bacterial infection / MGI
abnormal macrophage physiology / MGI
abnormal neutrophil physiology / MGI
granulomatous inflammation / MGI
glomerulonephritis / MGI
increased urine protein level / MGI
abnormal leukocyte adhesion / MGI
increased length of allograft survival / MGI
decreased susceptibility to experimental autoimmune encephalomyelitis / MGI
increased susceptibility to parasitic infection / MGI
glomerulosclerosis / MGI
abnormal renal glomerulus morphology / MGI
immune system phenotype / MGI
increased susceptibility to fungal infection / MGI
increased blood urea nitrogen level / MGI
decreased susceptibility to type III hypersensitivity reaction / MGI
increased susceptibility to type IV hypersensitivity reaction / MGI
decreased CD4-positive, alpha beta T cell number / MGI
decreased CD8-positive, alpha-beta T cell number / MGI
abnormal Kupffer cell morphology / MGI
increased IgG2a level / MGI
increased tumor necrosis factor secretion / MGI
decreased tumor necrosis factor secretion / MGI
increased interferon-gamma secretion / MGI
decreased circulating interferon-gamma level / MGI
decreased interleukin-10 secretion / MGI
increased interleukin-12 secretion / MGI
decreased interleukin-13 secretion / MGI
impaired neutrophil recruitment / MGI
abnormal chemokine secretion / MGI
cecum inflammation / MGI
decreased susceptibility to bacterial infection induced morbidity/mortality / MGI
abnormal cellular extravasation / MGI
abnormal circulating chemokine level / MGI
increased macrophage nitric oxide production / MGI
glomerular crescent / MGI
abnormal stria vascularis morphology / MGI
increased leukocyte cell number / MGI
increased neutrophil cell number / MGI
ruffled hair / MGI
cortical renal glomerulopathies / MGI
abnormal hepatocyte morphology / MGI
abnormal liver physiology / MGI
abnormal thyroid gland morphology / MGI
abnormal immune system morphology / MGI
lymphoid hyperplasia / MGI
enlarged spleen / MGI
enlarged lymph nodes / MGI
enlarged thymus / MGI
demyelination / MGI
abnormal skin condition / MGI
skin lesions / MGI
epidermal necrosis / MGI
increased body weight / MGI
impaired coordination / MGI
abnormal spatial learning / MGI
abnormal cardiovascular system physiology / MGI
abnormal apoptosis / MGI
focal hepatic necrosis / MGI
abnormal immune system physiology / MGI
decreased IgG level / MGI
abnormal thymus involution / MGI
decreased activated T cell number / MGI
autoimmune response / MGI
brain inflammation / MGI
conjunctivitis / MGI
kidney inflammation / MGI
vascular inflammation / MGI
vasculitis / MGI
salivary gland inflammation / MGI
increased tumor incidence / MGI
premature death / MGI
abnormal kidney morphology / MGI
abnormal kidney physiology / MGI
abnormal T cell differentiation / MGI
abnormal hypersensitivity reaction / MGI
abnormal gland morphology / MGI
no abnormal phenotype detected / MGI
abnormal lymph organ size / MGI
abnormal airway responsiveness / MGI
abnormal lymph node morphology / MGI
abnormal thymus medulla morphology / MGI
increased susceptibility to bacterial infection / MGI
increased susceptibility to viral infection / MGI
abnormal T cell physiology / MGI
increased immunoglobulin level / MGI
abnormal immunoglobulin level / MGI
increased IgG level / MGI
increased IgM level / MGI
increased IgA level / MGI
abnormal type III hypersensitivity reaction / MGI
abnormal immune system organ morphology / MGI
glomerulonephritis / MGI
albuminuria / MGI
joint swelling / MGI
increased urine protein level / MGI
increased circulating serum albumin level / MGI
decreased neuron apoptosis / MGI
thyroid inflammation / MGI
kidney failure / MGI
abnormal leukocyte adhesion / MGI
thymus atrophy / MGI
dilated renal glomerular capsule / MGI
increased autoantibody level / MGI
abnormal submandibular gland morphology / MGI
abnormal coronary artery morphology / MGI
abnormal strial intermediate cell morphology / MGI
abnormal stria vascularis vasculature morphology / MGI
abnormal conjunctival epithelium morphology / MGI
abnormal thyroid follicle morphology / MGI
increased anti-double stranded DNA antibody level / MGI
increased anti-single stranded DNA antibody level / MGI
increased anti-nuclear antigen antibody level / MGI
increased anti-histone antibody level / MGI
increased anti-erythrocyte antigen antibody level / MGI
increased susceptibility to systemic lupus erythematosus / MGI
decreased susceptibility to autoimmune diabetes / MGI
abnormal B cell morphology / MGI
increased spleen weight / MGI
increased thymus weight / MGI
abnormal T cell clonal deletion / MGI
decreased eosinophil cell number / MGI
increased lymphocyte cell number / MGI
increased B cell number / MGI
decreased B cell number / MGI
increased susceptibility to parasitic infection / MGI
abnormal cytotoxic T cell physiology / MGI
increased double-negative T cell number / MGI
abnormal T cell proliferation / MGI
liver hyperplasia / MGI
abnormal renal glomerulus morphology / MGI
abnormal myocardium layer morphology / MGI
increased susceptibility to autoimmune disorder / MGI
endocrine/exocrine gland phenotype / MGI
cardiovascular system phenotype / MGI
immune system phenotype / MGI
vision/eye phenotype / MGI
abnormal circulating protein level / MGI
abnormal T-helper 2 physiology / MGI
increased blood urea nitrogen level / MGI
increased circulating total protein level / MGI
abnormal enzyme/coenzyme activity / MGI
increased susceptibility to type III hypersensitivity reaction / MGI
increased apoptosis / MGI
decreased apoptosis / MGI
CNS inflammation / MGI
abnormal T cell morphology / MGI
increased NK cell number / MGI
decreased CD4-positive, alpha beta T cell number / MGI
decreased CD8-positive, alpha-beta T cell number / MGI
increased plasma cell number / MGI
lymph node hyperplasia / MGI
enlarged Peyer's patches / MGI
podocyte foot process effacement / MGI
increased follicular B cell number / MGI
abnormal marginal zone B cell morphology / MGI
increased marginal zone B cell number / MGI
decreased marginal zone B cell number / MGI
increased transitional stage B cell number / MGI
decreased transitional stage B cell number / MGI
decreased mature B cell number / MGI
increased immature B cell number / MGI
decreased immature B cell number / MGI
abnormal B cell activation / MGI
abnormal leukocyte morphology / MGI
increased gamma-delta T cell number / MGI
decreased spleen germinal center number / MGI
increased IgG1 level / MGI
increased IgG2a level / MGI
increased IgG2b level / MGI
increased IgG3 level / MGI
decreased interleukin-2 secretion / MGI
abnormal interleukin level / MGI
abnormal B cell receptor editing / MGI
decreased B cell apoptosis / MGI
abnormal splenic cell ratio / MGI
abnormal lymph node cell ratio / MGI
submandibular gland inflammation / MGI
lacrimal gland inflammation / MGI
decreased hepatocyte apoptosis / MGI
abnormal vagina weight / MGI
increased splenocyte number / MGI
increased sensitivity to induced morbidity/mortality / MGI
abnormal NK cell physiology / MGI
mortality/aging / MGI
decreased macrophage apoptosis / MGI
renal glomerulus hypertrophy / MGI
abnormal glomerular capillary morphology / MGI
abnormal renal glomerulus basement membrane morphology / MGI
expanded mesangial matrix / MGI
increased renal glomerulus apoptosis / MGI
renal cast / MGI
increased renal glomerulus basement membrane thickness / MGI
mesangial cell hyperplasia / MGI
increased kidney cell proliferation / MGI
abnormal glomerular capillary endothelium morphology / MGI
glomerular crescent / MGI
increased or absent threshold for auditory brainstem response / MGI
lacrimal gland atrophy / MGI
thymus cortex atrophy / MGI
impaired leukocyte tethering or rolling / MGI
renal glomerular protein deposits / MGI
renal glomerular immunoglobulin deposits / MGI

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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)
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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MRL.Cg-Ccr1tm1Gao Faslpr/Cnbc