C57Bl/6.CAST-Cdh23Ahl+-AOX-Rosa26

Status	Available to order
EMMA ID	EM:15605
International strain name	C57Bl/6.CAST-Cdh23Ahl+-AOX-Rosa26
Alternative name	C57Bl/6.CAST-Cdh23Ahl+-AOX-Rosa26
Strain type	Targeted Mutant Strains : Knock-in
Allele/Transgene symbol	Gt(ROSA)26Sor^{tm1.1(CAG-AOX)Htj}, Cdh23^Ahl+
Gene/Transgene symbol	Gt(ROSA)26Sor, Cdh23

Information from provider

Provider	Georg Klump
Provider affiliation	Department of Neuroscience, Carl von Ossietzky University Oldenburg
Additional owner	Dr. Marten Szibor, BioMediTech & Tampere University Hospital, Faculty of Medicine and Health Technology, FI-33014 Tampere University, Finland.
Genetic information	An expression cassette for alternative oxidase (AOX) from Ciona intestinalis targeting the mouse Gt(ROSA)26Sor locus was introduced into the genome of v6.5 mouse embryonic stem cells by homologous recombination. AOX expression is controlled by a CAG promoter (MGI:5824337 allele ID). The ES cells were propagated and injected into blastocyst-stage embryos in the laboratory of Prof. Thomas Braun at the Max Planck Institute for Heart and Lung Research in Bad Nauheim. Mice were backcrossed to the C57BL/6J background. This strain is only available as frozen sperm. It is recommended to use B6.CAST-Cdh23Ahl+/Kjn (available from The Jackson Laboratory) as oocyte donors for rederivation and afterwards produce homozygous AOX knock-in mice by intercrossing. In case the rederivation is done in the C57BL/6J background, the recipient will need to produce homozygous mice (i.e. homzoygous for AOX knock-in and for B6.CAST-Cdh23Ahl+ background), by appropriate crossing within the F1 progeny.
Phenotypic information	Homozygous: No obvious phenotype. Heterozygous: No obvious phenotype.
Breeding history	A minimum of eight generations of half- and/or sib matings have been performed with mice with knock-in of AOX on C57BL/6.CAST-Cdh23^Ahl+ background.
References	Broad AOX expression in a genetically tractable mouse model does not disturb normal physiology.;Szibor Marten, Dhandapani Praveen K, Dufour Eric, Holmström Kira M, Zhuang Yuan, Salwig Isabelle, Wittig Ilka, Heidler Juliana, Gizatullina Zemfira, Gainutdinov Timur, null null, Fuchs Helmut, Gailus-Durner Valérie, de Angelis Martin Hrabě, Nandania Jatin, Velagapudi Vidya, Wietelmann Astrid, Rustin Pierre, Gellerich Frank N, Jacobs Howard T, Braun Thomas, ;2017;Disease models & mechanisms;10;163-171; 28067626
Homozygous fertile	yes
Homozygous viable	yes
Homozygous matings required	yes
Immunocompromised	no

Information from EMMA

Archiving centre	Helmholtz Zentrum Muenchen - German Research Center for Environmental Health (GmbH), Oberschleißheim, Germany
Animals used for archiving	homozygous 0

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Usher syndrome type 1 / Orphanet_231169
Autosomal recessive non-syndromic sensorineural deafness type DFNB / Orphanet_90636

IMPC phenotypes (gene matching)

abnormal posture / IMPC
decreased circulating calcium level / IMPC
preweaning lethality, incomplete penetrance / IMPC
absent pinna reflex / IMPC
decreased body length / IMPC
decreased circulating glucose level / IMPC
increased mean corpuscular hemoglobin / IMPC
trunk curl / IMPC
increased lean body mass / IMPC
decreased total body fat amount / IMPC
increased circulating sodium level / IMPC
abnormal gait / IMPC
impaired righting response / IMPC
decreased fasting circulating glucose level / IMPC
increased circulating chloride level / IMPC
stereotypic behavior / IMPC
decreased leukocyte cell number / IMPC
increased bone mineral content / IMPC

MGI phenotypes (gene matching)

abnormal ear position / MGI
abnormal inner ear morphology / MGI
abnormal cochlea morphology / MGI
abnormal organ of Corti morphology / MGI
organ of Corti degeneration / MGI
abnormal stria vascularis morphology / MGI
tremors / MGI
abnormal maternal nurturing / MGI
abnormal stationary movement / MGI
abnormal locomotor behavior / MGI
ataxia / MGI
circling / MGI
bidirectional circling / MGI
hyperactivity / MGI
abnormal gait / MGI
head bobbing / MGI
decreased grooming behavior / MGI
abnormal startle reflex / MGI
decreased startle reflex / MGI
impaired swimming / MGI
decreased litter size / MGI
abnormal reflex / MGI
abnormal hearing physiology / MGI
deafness / MGI
abnormal motor capabilities/coordination/movement / MGI
abnormal ear morphology / MGI
abnormal cochlear hair cell morphology / MGI
head shaking / MGI
decreased vertical activity / MGI
abnormal cochlear ganglion morphology / MGI
cochlear ganglion degeneration / MGI
abnormal otolith morphology / MGI
abnormal response to novelty / MGI
abnormal cochlear sensory epithelium morphology / MGI
cochlear ganglion hypoplasia / MGI
abnormal defecation / MGI
abnormal ear physiology / MGI
vestibular saccular degeneration / MGI
vestibular saccular macula degeneration / MGI
cochlear hair cell degeneration / MGI
stria vascularis degeneration / MGI
decreased cochlear inner hair cell number / MGI
cochlear inner hair cell degeneration / MGI
abnormal cochlear outer hair cell morphology / MGI
decreased cochlear outer hair cell number / MGI
cochlear outer hair cell degeneration / MGI
decreased cochlear hair cell number / MGI
abnormal cochlear nerve compound action potential / MGI
abnormal hair cell mechanoelectric transduction / MGI
abnormal cochlear hair cell physiology / MGI
abnormal cochlear outer hair cell physiology / MGI
abnormal orientation of outer hair cell stereociliary bundles / MGI
abnormal orientation of inner hair cell stereociliary bundles / MGI
abnormal vestibular hair cell stereociliary bundle morphology / MGI
abnormal cochlear hair cell stereociliary bundle morphology / MGI
abnormal orientation of cochlear hair cell stereociliary bundles / MGI
decreased cochlear hair cell stereocilia number / MGI
abnormal outer hair cell stereociliary bundle morphology / MGI
decreased outer hair cell stereocilia number / MGI
absent outer hair cell stereocilia / MGI
abnormal inner hair cell stereociliary bundle morphology / MGI
abnormal cochlear hair cell inter-stereocilial links morphology / MGI
abnormal cochlear hair bundle tip links morphology / MGI
increased susceptibility to noise-induced hearing loss / MGI
abnormal distortion product otoacoustic emission / MGI
absent distortion product otoacoustic emissions / MGI
abnormal auditory brainstem response / MGI
abnormal vestibular system physiology / MGI
increased susceptibility to age-related hearing loss / MGI
absent linear vestibular evoked potential / MGI
head tilt / MGI
head tossing / MGI
novel environmental response-related retropulsion / MGI
homeostasis/metabolism phenotype / MGI
hearing/vestibular/ear phenotype / MGI
behavior/neurological phenotype / MGI
vision/eye phenotype / MGI
abnormal eye electrophysiology / MGI
impaired hearing / MGI
absent pinna reflex / MGI
absent startle reflex / MGI
abnormal inner hair cell kinocilium morphology / MGI
abnormal outer hair cell kinocilium morphology / MGI
increased or absent threshold for auditory brainstem response / MGI
decreased a wave implicit time / MGI
decreased b wave implicit time / MGI
abnormal ear pigmentation / MGI
decreased circulating LDL cholesterol level / MGI
decreased hematocrit / MGI
increased leukocyte cell number / MGI
decreased neutrophil cell number / MGI
extramedullary hematopoiesis / MGI
abnormal erythropoiesis / MGI
abnormal myocardial fiber morphology / MGI
abnormal cell death / MGI
increased granulocyte number / MGI
increased cell proliferation / MGI
alopecia / MGI
short snout / MGI
abnormal digestive system morphology / MGI
gastrointestinal hemorrhage / MGI
increased rib number / MGI
abnormal pulmonary artery morphology / MGI
rectal prolapse / MGI
abnormal renal/urinary system morphology / MGI
abnormal mammary gland development / MGI
abnormal spleen morphology / MGI
enlarged spleen / MGI
enlarged lymph nodes / MGI
dystrophic muscle / MGI
absent notochord / MGI
abnormal retinal photoreceptor morphology / MGI
abnormal neuromuscular synapse morphology / MGI
pigmentation phenotype / MGI
increased body weight / MGI
decreased body weight / MGI
decreased body size / MGI
retinal degeneration / MGI
disorganized retinal layers / MGI
abnormal optic nerve morphology / MGI
decreased anxiety-related response / MGI
circling / MGI
hypoactivity / MGI
impaired coordination / MGI
abnormal eating behavior / MGI
limb grasping / MGI
increased circulating triglyceride level / MGI
increased circulating free fatty acid level / MGI
increased circulating HDL cholesterol level / MGI
hyperglycemia / MGI
anemia / MGI
abnormal blood vessel morphology / MGI
abnormal mesoderm development / MGI
abnormal somite development / MGI
decreased trophoblast giant cell number / MGI
postnatal growth retardation / MGI
impaired wound healing / MGI
abnormal humoral immune response / MGI
arrested B cell differentiation / MGI
arrested T cell differentiation / MGI
increased inflammatory response / MGI
reduced fertility / MGI
reduced female fertility / MGI
male infertility / MGI
decreased litter size / MGI
respiratory distress / MGI
abnormal pain threshold / MGI
increased thermal nociceptive threshold / MGI
abnormal coat/hair pigmentation / MGI
abnormal glucose homeostasis / MGI
increased circulating insulin level / MGI
premature death / MGI
abnormal tail morphology / MGI
abnormal kidney morphology / MGI
abnormal neural tube morphology / MGI
no abnormal phenotype detected / MGI
abnormal seminiferous tubule morphology / MGI
small lymph nodes / MGI
abnormal spleen white pulp morphology / MGI
abnormal bone marrow cell morphology/development / MGI
abnormal megakaryocyte progenitor cell morphology / MGI
abnormal proerythroblast morphology / MGI
abnormal megakaryocyte morphology / MGI
increased mean corpuscular volume / MGI
decreased mean corpuscular volume / MGI
increased mean platelet volume / MGI
hepatic steatosis / MGI
abnormal epididymis morphology / MGI
vestigial tail / MGI
reticulocytosis / MGI
abnormal keratinocyte differentiation / MGI
decreased circulating corticosterone level / MGI
asthenozoospermia / MGI
oligozoospermia / MGI
abnormal renal tubule morphology / MGI
decreased circulating insulin level / MGI
abnormal chemical nociception / MGI
dilated heart left ventricle / MGI
decreased vertical activity / MGI
abnormal notochord morphology / MGI
abnormal ocular fundus morphology / MGI
albuminuria / MGI
decreased hemoglobin content / MGI
decreased erythrocyte cell number / MGI
no phenotypic analysis / MGI
vertebral transformation / MGI
abnormal lumbar vertebrae morphology / MGI
abnormal skeletal muscle fiber morphology / MGI
absent allantois / MGI
hypospadia / MGI
anal atresia / MGI
increased erythroid progenitor cell number / MGI
abnormal embryonic hematopoiesis / MGI
kinked neural tube / MGI
epididymal inflammation / MGI
nervous system phenotype / MGI
abnormal nervous system morphology / MGI
kidney cysts / MGI
pallor / MGI
abnormal neural tube closure / MGI
abnormal retinal inner nuclear layer morphology / MGI
vascular smooth muscle hypoplasia / MGI
skeletal muscle necrosis / MGI
abnormal defecation / MGI
abnormal heart left ventricle morphology / MGI
abnormal circulating lipid level / MGI
abnormal hormone level / MGI
increased lean body mass / MGI
abnormal rod electrophysiology / MGI
abnormal cone electrophysiology / MGI
caudal body truncation / MGI
abnormal vitelline vascular remodeling / MGI
abnormal sarcomere morphology / MGI
increased compact bone thickness / MGI
transmission ratio distortion / MGI
abnormal basement membrane morphology / MGI
short frontal bone / MGI
short nasal bone / MGI
increased incidence of tumors by chemical induction / MGI
caudal vertebral transformation / MGI
cervical vertebral transformation / MGI
lumbar vertebral transformation / MGI
thoracic vertebral transformation / MGI
cervical vertebral fusion / MGI
absent caudal vertebrae / MGI
small sacral vertebrae / MGI
abnormal vertebral column morphology / MGI
notochord degeneration / MGI
truncated notochord / MGI
abnormal platelet morphology / MGI
decreased susceptibility to experimental autoimmune encephalomyelitis / MGI
increased hematopoietic stem cell number / MGI
decreased testis weight / MGI
increased energy expenditure / MGI
decreased adiponectin level / MGI
decreased epididymis weight / MGI
abnormal epididymis epithelium morphology / MGI
skin inflammation / MGI
decreased B-1 B cell number / MGI
increased neuronal precursor cell number / MGI
increased osteoblast cell number / MGI
decreased lymphocyte cell number / MGI
decreased cardiac muscle contractility / MGI
cachexia / MGI
increased susceptibility to injury / MGI
decreased circulating cholesterol level / MGI
abnormal retinal pigment epithelium morphology / MGI
abnormal rostral-caudal axis patterning / MGI
glomerulosclerosis / MGI
increased oxygen consumption / MGI
impaired glucose tolerance / MGI
spina bifida occulta / MGI
decreased triglyceride level / MGI
abnormal renal glomerulus morphology / MGI
insulin resistance / MGI
muscle phenotype / MGI
homeostasis/metabolism phenotype / MGI
growth/size/body region phenotype / MGI
endocrine/exocrine gland phenotype / MGI
cardiovascular system phenotype / MGI
immune system phenotype / MGI
reproductive system phenotype / MGI
skeleton phenotype / MGI
hematopoietic system phenotype / MGI
increased circulating thyroxine level / MGI
increased circulating triiodothyronine level / MGI
abnormal skeleton morphology / MGI
abnormal body temperature / MGI
choroidal neovascularization / MGI
abnormal eye electrophysiology / MGI
decreased circulating glucose level / MGI
increased mean corpuscular hemoglobin / MGI
decreased mean corpuscular hemoglobin / MGI
decreased angiogenesis / MGI
increased bone mass / MGI
abnormal cell physiology / MGI
increased susceptibility to diet-induced obesity / MGI
decreased susceptibility to diet-induced obesity / MGI
decreased circulating leptin level / MGI
increased circulating leptin level / MGI
increased mitochondrial fission / MGI
increased apoptosis / MGI
abnormal tail development / MGI
abnormal spermatid morphology / MGI
intestine polyps / MGI
abnormal paraxial mesoderm morphology / MGI
abnormal podocyte slit diaphragm morphology / MGI
decreased CD4-positive, alpha beta T cell number / MGI
decreased CD8-positive, alpha-beta T cell number / MGI
abnormal granulocyte differentiation / MGI
abnormal monocyte differentiation / MGI
podocyte foot process effacement / MGI
increased germinal center B cell number / MGI
decreased B-2 B cell number / MGI
decreased mature B cell number / MGI
increased megakaryocyte cell number / MGI
abnormal bone ossification / MGI
retinal photoreceptor degeneration / MGI
abnormal medium spiny neuron morphology / MGI
retinal outer nuclear layer degeneration / MGI
increased circulating tumor necrosis factor level / MGI
increased circulating interleukin-1 alpha level / MGI
increased circulating interleukin-1 beta level / MGI
increased interleukin-17 secretion / MGI
decreased survivor rate / MGI
increased B cell apoptosis / MGI
increased liver iron level / MGI
increased spleen iron level / MGI
increased circulating iron level / MGI
lipofuscinosis / MGI
abnormal physiological response to xenobiotic / MGI
decreased erythroid progenitor cell number / MGI
anal stenosis / MGI
hairpin sperm flagellum / MGI
increased abdominal fat pad weight / MGI
increased trophoblast giant cell number / MGI
centrally nucleated skeletal muscle fibers / MGI
skeletal muscle endomysial fibrosis / MGI
abnormal synaptonemal complex / MGI
abnormal mammary gland duct morphology / MGI
decreased thymocyte apoptosis / MGI
enlarged popliteal lymph nodes / MGI
decreased birth body size / MGI
impaired somite development / MGI
increased total body fat amount / MGI
abnormal placenta physiology / MGI
abnormal retinal blood vessel morphology / MGI
decreased mammary gland epithelial cell proliferation / MGI
decreased tumor latency / MGI
altered tumor pathology / MGI
mortality/aging / MGI
integument phenotype / MGI
increased bone volume / MGI
abnormal double-strand DNA break repair / MGI
neonatal lethality, complete penetrance / MGI
perinatal lethality, incomplete penetrance / MGI
prenatal lethality, complete penetrance / MGI
embryonic lethality during organogenesis, complete penetrance / MGI
preweaning lethality, complete penetrance / MGI
prenatal lethality, incomplete penetrance / MGI
embryonic lethality during organogenesis, incomplete penetrance / MGI
abnormal anterior visceral endoderm cell migration / MGI
increased circulating erythropoietin level / MGI
renal tubule atrophy / MGI
lethality, complete penetrance / MGI
increased mitochondria number / MGI
rectal atresia / MGI
rectourethral fistula / MGI
increased hematopoietic stem cell proliferation / MGI
increased food intake / MGI
impaired mammary gland growth during pregnancy / MGI
decreased paraxial mesoderm size / MGI
abnormal skeletal muscle regeneration / MGI
testis degeneration / MGI
preputial gland inflammation / MGI
nervous system inclusion bodies / MGI
decreased fatty acid beta-oxidation / MGI
epididymis hypertrophy / MGI
spermatocele / MGI
epididymis fibrosis / MGI
increased epididymal cell proliferation / MGI
increased epididymal epithelium cell proliferation / MGI
increased heart iron level / MGI
increased intestinal iron level / MGI

Literature references

Broad AOX expression in a genetically tractable mouse model does not disturb normal physiology.;Szibor Marten, Dhandapani Praveen K, Dufour Eric, Holmström Kira M, Zhuang Yuan, Salwig Isabelle, Wittig Ilka, Heidler Juliana, Gizatullina Zemfira, Gainutdinov Timur, null null, Fuchs Helmut, Gailus-Durner Valérie, de Angelis Martin Hrabě, Nandania Jatin, Velagapudi Vidya, Wietelmann Astrid, Rustin Pierre, Gellerich Frank N, Jacobs Howard T, Braun Thomas, ;2017;Disease models & mechanisms;10;163-171; 28067626

Information on how we integrate external resources can be found here

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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^*

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

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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