B6.129(Cg)-Pax3^{tm3.1(Pax7)Buck} Pax7^tm2Pgr/Orl

Status	Available to order
EMMA ID	EM:05737
Citation information	RRID:IMSR_EM:05737 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	B6.129(Cg)-Pax3^{tm3.1(Pax7)Buck} Pax7^tm2Pgr/Orl
Alternative name	B6.Pax3Pax7/+ ; Pax7LacZ/+
Strain type	Targeted Mutant Strains : Knock-in
Allele/Transgene symbol	Pax3^{tm3.1(Pax7)Buck}, Pax7^tm2Pgr
Gene/Transgene symbol	Pax3, Pax7

Information from provider

Provider	Frederic RELAIX
Provider affiliation	Groupe Myologie, UMR-S 787 - INSERM - UPMC-Paris VI - Institut de Myologie
Additional owner	Margaret Buckingham, Molecular Genetics of Development (URA CNRS 2578), Institut Pasteur, Paris, France
Genetic information	The Pax7 gene has been introduced in the Pax3 locus and the lacZ gene has been introduced in the Pax7 locus. Targeting construct is derived from 129 DNA.
Phenotypic information	The double heterozygous mice are viable, fertile and present a white belly spot. The double homozygotes are not viable: homozygous embryo die at birth.
Breeding history	Double heterozygotes are viable and fertile. Double homozygotes are not viable. The heterozygous male mice of each line are crossed. The line has been backcrossed to C57BL/6 background for at least 5 generations.
References	Divergent functions of murine Pax3 and Pax7 in limb muscle development.;Relaix Frédéric, Rocancourt Didier, Mansouri Ahmed, Buckingham Margaret, ;2004;Genes & development;18;1088-105; 15132998
Homozygous fertile	no
Homozygous viable	no
Homozygous matings required	no
Immunocompromised	no

Information from EMMA

Archiving centre	CNRS-TAAM – Typing and Archiving of Animal Models, Orléans, France
Animals used for archiving	homozygous C57BL/6J males, heterozygous C57BL/6J females
Breeding at archiving centre	Pax3Pax7/Pax7 (Pax3-tm3.1(Pax7)Buck/Pax7-tm2Pgr) homozygous females mated with Pax7LacZ (Pax7-tm2Pgr) heterozygous males.

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Craniofacial-deafness-hand syndrome / Orphanet_1529
Waardenburg syndrome type 1 / Orphanet_894
Waardenburg syndrome type 3 / Orphanet_896

IMPC phenotypes (gene matching)

preweaning lethality, complete penetrance / IMPC
decreased circulating triglyceride level / IMPC
preweaning lethality, incomplete penetrance / IMPC

MGI phenotypes (allele matching)

decreased cell proliferation / MGI
abnormal myogenesis / MGI
spina bifida / MGI
integument phenotype / MGI
perinatal lethality, complete penetrance / MGI
abnormal diaphragm development / MGI
abnormal muscle development / MGI
abnormal muscle precursor cell migration / MGI
nervous system phenotype / MGI
embryo phenotype / MGI
short maxilla / MGI
pointed snout / MGI
abnormal small intestine morphology / MGI
postnatal growth retardation / MGI
abnormal nasal capsule morphology / MGI
abnormal serous gland morphology / MGI
postnatal lethality, incomplete penetrance / MGI
abnormal muscle regeneration / MGI
decreased satellite cell number / MGI

MGI phenotypes (gene matching)

thin ventricular wall / MGI
abnormal interventricular septum morphology / MGI
double outlet right ventricle / MGI
decreased cell proliferation / MGI
diluted coat color / MGI
belly spot / MGI
abnormal hindlimb morphology / MGI
kinked tail / MGI
abnormal thyroid gland morphology / MGI
abnormal thymus morphology / MGI
abnormal myogenesis / MGI
abnormal muscle development / MGI
abnormal myotome development / MGI
abnormal skeletal muscle morphology / MGI
thin diaphragm muscle / MGI
abnormal brain ventricle morphology / MGI
abnormal lateral ventricle morphology / MGI
abnormal hindbrain morphology / MGI
exencephaly / MGI
incomplete rostral neuropore closure / MGI
open neural tube / MGI
small embryonic telencephalon / MGI
abnormal spinal cord morphology / MGI
abnormal dorsal root ganglion morphology / MGI
disorganized dorsal root ganglion / MGI
absent skin pigmentation / MGI
decreased body size / MGI
cyanosis / MGI
abnormal somite development / MGI
failure to gastrulate / MGI
decreased embryo size / MGI
respiratory distress / MGI
neoplasm / MGI
abnormal coat/hair pigmentation / MGI
abnormal limb morphology / MGI
abnormal tail morphology / MGI
abnormal neural tube morphology / MGI
no abnormal phenotype detected / MGI
abnormal pulmonary alveolus morphology / MGI
abnormal thymus lobule morphology / MGI
abnormal semicircular canal morphology / MGI
delayed neural tube closure / MGI
persistent truncus arteriosis / MGI
abnormal pharyngeal arch artery morphology / MGI
dilated heart left ventricle / MGI
dilated heart right ventricle / MGI
white spotting / MGI
head spot / MGI
variable body spotting / MGI
abnormal neural crest cell migration / MGI
small thyroid gland / MGI
ectopic thymus / MGI
no phenotypic analysis / MGI
curly tail / MGI
spina bifida / MGI
abnormal muscle precursor cell migration / MGI
decreased cochlear coiling / MGI
nervous system phenotype / MGI
abnormal midbrain development / MGI
abnormal tongue muscle morphology / MGI
retroesophageal right subclavian artery / MGI
abnormal dermomyotome development / MGI
abnormal myocardial fiber physiology / MGI
abnormal bony labyrinth / MGI
increased mitotic index / MGI
abnormal neural fold elevation formation / MGI
absent skeletal muscle / MGI
absent ultimobranchial body / MGI
absent coat pigmentation / MGI
absent thyroid gland / MGI
hearing/vestibular/ear phenotype / MGI
embryo phenotype / MGI
cardiovascular system phenotype / MGI
vision/eye phenotype / MGI
hypopigmentation / MGI
decreased ventricle muscle contractility / MGI
abnormal endolymphatic duct morphology / MGI
abnormal otic vesicle development / MGI
abnormal vena cava morphology / MGI
abnormal vestibular saccule morphology / MGI
abnormal utricle morphology / MGI
abnormal cardiac outflow tract development / MGI
congestive heart failure / MGI
absent hypaxial muscle / MGI
abnormal sixth pharyngeal arch artery morphology / MGI
short endolymphatic duct / MGI
abnormal ventral coat pigmentation / MGI
absent dorsal root ganglion / MGI
embryonic lethality / MGI
caudal rachischisis / MGI
abnormal tail hair pigmentation / MGI
abnormal hind foot hair pigmentation / MGI
variable depigmentation / MGI
transverse fur striping / MGI
ventricular septal defect / MGI
muscular ventricular septal defect / MGI
persistent right dorsal aorta / MGI
common truncal valve / MGI
patent tricuspid valve / MGI
dilated pulmonary trunk / MGI
thick interventricular septum / MGI
integument phenotype / MGI
postnatal lethality, complete penetrance / MGI
neonatal lethality, incomplete penetrance / MGI
perinatal lethality, complete penetrance / MGI
prenatal lethality, complete penetrance / MGI
embryonic lethality between implantation and somite formation, complete penetrance / MGI
embryonic lethality during organogenesis, complete penetrance / MGI
lethality throughout fetal growth and development, complete penetrance / MGI
prenatal lethality, incomplete penetrance / MGI
embryonic lethality, incomplete penetrance / MGI
embryonic lethality during organogenesis, incomplete penetrance / MGI
lethality throughout fetal growth and development, incomplete penetrance / MGI
decreased tail pigmentation / MGI
abnormal diaphragm development / MGI
spina bifida cystica / MGI
increased embryonic neuroepithelium apoptosis / MGI
abnormal common carotid artery morphology / MGI
abnormal mammary line morphology / MGI
abnormal mammary placode morphology / MGI
abnormal embryo morphology / MGI
abnormal hypoglossal cord morphology / MGI
short maxilla / MGI
pointed snout / MGI
abnormal small intestine morphology / MGI
muscle weakness / MGI
abnormal muscle regeneration / MGI
thin diaphragm muscle / MGI
decreased body size / MGI
postnatal growth retardation / MGI
premature death / MGI
no abnormal phenotype detected / MGI
no phenotypic analysis / MGI
nervous system phenotype / MGI
decreased satellite cell number / MGI
myositis / MGI
abnormal nasal capsule morphology / MGI
decreased skeletal muscle mass / MGI
cachexia / MGI
muscle phenotype / MGI
embryo phenotype / MGI
digestive/alimentary phenotype / MGI
cellular phenotype / MGI
abnormal serous gland morphology / MGI
decreased skeletal muscle fiber diameter / MGI
skeletal muscle fibrosis / MGI
decreased birth body size / MGI
decreased skeletal muscle weight / MGI
postnatal lethality, incomplete penetrance / MGI

Literature references

Divergent functions of murine Pax3 and Pax7 in limb muscle development.;Relaix Frédéric, Rocancourt Didier, Mansouri Ahmed, Buckingham Margaret, ;2004;Genes & development;18;1088-105; 15132998

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

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Practical information

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Material Transfer Agreement (MTA)
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B6.129(Cg)-Pax3tm3.1(Pax7)Buck Pax7tm2Pgr/Orl