JAX Mice Database - 006562 B6.CBy(Cg)-Gusb<mps> Gpi1<a-m1J>/BrkJ

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Strain Name:	B6.CBy(Cg)-Gusb^mps Gpi1^a-m1J/BrkJ
Stock Number:	006562
Availability:	Repository- Live

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General Terms and Conditions

Former Name	B6.Cg-Gusb^mps Gpi1^a-m1Ehs/BrkJ (Changed: 20-DEC-07 )
	B6.Cg-Gusb^mps Gpi1^c/SopJ (Changed: 25-JAN-07 )
Genes & Alleles	Gpi1; Gpi1^a-m1J; Gusb; Gusb^mps;

Product Information

Strain Details

Type JAX^® GEMM^® Strain - Congenic

Additional information on JAX^® GEMM^® Strains.

Type JAX^® GEMM^® Strain - Mutant Strain

Type JAX^® GEMM^® Strain - Spontaneous Mutation

Mating System See Colony Maintenance (Female x Male)

Species laboratory mouse

Background Strain C57BL/6J

H2 Haplotype b

Generation N11+F3pF1+N1 (15-APR-08)

Appearance
black
Related Genotype: a/a
Strain Description
Mice homozygous for the "mps" (mucopolysaccharidosis type VII or MPS VII) mutation are devoid of expression of the lysosomal enzyme beta glucuronidase. Homozygous animals are viable, but females have a deficiency in lactation. Skeletal and connective tissue anomalies in both males and females are believed to prevent successful breeding. As this mutation is recessive, heterozygous mice are phenotypically similar to wildtype. Homozygotes exhibit short and thickened long bones (smaller than heterozygous or wildtype littermates), "pug type" appearance of the nose, hepatomegaly, splenomegaly, corneal clouding, and deafness. MPS VII mice are a model of the beta glucuronidase enzyme deficiency in humans called Sly Disease. They may be useful in developing new therapies (enzyme replacement, cell transplantation, gene therapy) broadly applicable to other lysosomal storage diseases.
Strain Development
This strain was generated by crossing B6.CBy(Cg)-Gpi1^a-m1J/BrkJ (Stock No. 006561) and B6.Cg-Gusb^mps/BrkJ (Stock No. 006407). Progeny with the appropriate alleles were backcrossed with C57BL/6 for 10 generations by Dr. Jane Barker at The Jackson Laboratory.

Mammalian Phenotype Terms assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX^® Mice strain.
Gusb^mps/Gusb^mps
involves: C57BL/6By
life span-post-weaning/aging

premature death (MGI Ref ID J:13923)

death between 150 and 200 days

adipose tissue phenotype

decreased white adipose tissue amount (MGI Ref ID J:13923)

absent, but normal brown fat amount

craniofacial phenotype

short snout (MGI Ref ID J:13923)

endocrine/exocrine gland phenotype

abnormal lactation (MGI Ref ID J:13923)

inadequate lactation to nurture pups

growth/size phenotype

decreased body length (MGI Ref ID J:13923)

dwarfism (MGI Ref ID J:13923)

subtle

homeostasis/metabolism phenotype

decreased circulating cholesterol level (MGI Ref ID J:13923)

compared to littermate controls

limbs/digits/tail phenotype

short limbs (MGI Ref ID J:13923)

short tail (MGI Ref ID J:13923)

reproductive system phenotype

abnormal lactation (MGI Ref ID J:13923)

inadequate lactation to nurture pups

male infertility (MGI Ref ID J:13923)

sterility not due to reproductive or gonadal tract dysmorphology or to sperm numbers, morphology, or motility

skeleton phenotype

abnormal skeleton morphology (MGI Ref ID J:13923)

mutant bones shorter and thicker than littermate controls, but contain same amount of mineralization

Gene & Allele Details

Allele Symbol		Gpi1^a-m1J
Allele Name		mutant 1, Jackson
Common Name(s)		Gpi1^c-J;
Strain of Origin		CByJ.Cg-Foxn1^nu/J
Gene Symbol and Name		Gpi1, glucose phosphate isomerase 1
Chromosome		7
Gene Common Name(s)		AMF; GNPI; Gpi-1; Gpi-1r; Gpi-1s; Gpi-1t; Gpi1-r; Gpi1-s; Gpi1-t; NLK; Org; PGI; PHI; SA-36; glucose phosphate isomerase 1, regulatory; glucose phosphate isomerase 1, structural; glucose phosphate isomerase 1, temporal;
Molecular Note		In 2000, a mouse of BALB/cByJ background from a production colony at The Jackson Laboratory (TJL) was subjected to isoenzyme analysis in the TJL Quality Control Laboratory and found to have glucose phosphate isomerase 1 activity associated with an electrophoretic band of fast mobility identical to that of GPI1^c, rather than the expected slower mobility of GPI1^a.

Allele Symbol		Gusb^mps
Allele Name		beta glucuronidase, mucopolysaccharidosis VII
Common Name(s)		Gus-b; asd; gus^mps;
Mutation Made By		Brian Soper, The Jackson Laboratory
Strain of Origin		C57BL/6By
Gene Symbol and Name		Gusb, glucuronidase, beta
Chromosome		5
Gene Common Name(s)		AI747421; Ac2-223; FLJ39445; Gur; Gus; Gus-r; Gus-s; Gus-t; Gus-u; Gut; MPS7; adipose storage deficiency; asd; beta-glucuronidase regulator; beta-glucuronidase structural; beta-glucuronidase systemic regulator; beta-glucuronidase temporal; expressed sequence AI747421; g;
Molecular Note		A 1-bp deletion creates a frameshift mutation within exon 10, which introduces a premature stop codon at codon 497. [MGI Ref ID J:13207]

Control Information

	Control
	000664 C57BL/6J

Considerations for Choosing Controls

Colony Maintenance

Breeding & Husbandry	When maintaining a live colony, mice heterozygous for the Gusb^mps allele and homozygous for the Gpi1^a-m1J allele are bred together. Mice homozygous for the Gusb^mps recessive mutation are viable, but exhibit breeding problems.
Diet Information	LabDiet® 5K52/5K67

Related Strains

Strains carrying Gpi1^a-m1J allele

006561 B6.CBy(Cg)-Gpi1^a-m1J/BrkJ

006560 CBy(Cg)-Gpi1^a-m1J/BrkJ

View Strains carrying Gpi1^a-m1J (2 strains)

Strains carrying Gusb^mps allele

006564 B6(C)-Kit^W-41J Gusb^mps/BrkJ

006559 B6.C-H2^bm1/ByBir-Gusb^mps/BrkJ

000256 B6.C-H2^bm1/ByBir-Gusb^mps/J

006407 B6.Cg-Gusb^mps/BrkJ

002086 B6.Cg-Gusb^mps Tg(Gus^sx)1Wat/J

005053 NOD.Cg-Prkdc^scid Gusb^mps/SndsJ

001880 STOCK Gusb^mps Tg(GUSB)4Sly/BirJ

View Strains carrying Gusb^mps (7 strains)

Strains carrying other alleles of Gpi1

005730 129S6.CB(B6)-Del(1)1Brk Gpi1^b/Gpi1^c/BrkMdfJ

002027 129S8/SvEv-Gpi1^c Hprt1^b-m2/J

000600 B6-Gpi1^b x B6CBCa A^w-J/A-T(7;15)9H Gpi1^a/J

000377 B6.C-H22^c Gpi1^a/(HW38)ByJ

000123 B6.C-H24^c Gpi1^a/(HW54)ByJ

001622 B6.CAST-Gpi1^a.Cg-Hba^th-J

000797 B6.CAST-Gpi1^a/EiJ

002074 B6.Cg-Gpi1^a Hbb^d H1^b/DehJ

001317 B6.Cg-Igh^a Thy1^a Gpi1^a/J

000909 B6CBy-Gpi1^a/Gpi1^b/J or Gpi1^a-m1/Gpi1^b/J

002800 C3.Cg-Gpi1^a/J

003585 NOD.B6-(Gpi1-D7Mit346)/LtJ

View Strains carrying other alleles of Gpi1 (12 strains)

Strains carrying other alleles of Gusb

005643 B6.129X-Gusb^tm1Sly/J

005644 B6.129X-Gusb^tm3Sly/J

006557 B6.C3-Gusb^mps-2J/BrkJ

006558 B6.Cg-H2^bm1 Tg(GUSB)4Sly/SndsJ

003525 C3H/HeOuJ-Gusb^mps-2J/BrkJ

005322 C57BL/6J-Gusb^mps-3J/J

001880 STOCK Gusb^mps Tg(GUSB)4Sly/BirJ

View Strains carrying other alleles of Gusb (7 strains)

Additional Web Information

Congenic Nomenclature

Animal Health Reports

Room Number AX12

Research Applications

This mouse can be used to support research in many areas including:

Research Tools
Genetics Research (Tissue/Cell Markers)

Gusb^mps related

Developmental Biology Research
Growth Defects Growth Defects (homozygous)
Skeletal Defects

Metabolism Research

Mouse/Human Gene Homologs
mucopolysaccharidosis type VII, GUSB deficiency

Neurobiology Research
Behavioral and Learning Defects

Sensorineural Research

References

Additional References

Price and Supply Information

Strain Name:	*B6.CBy(Cg)-Gusb^mps Gpi1^a-m1J/BrkJ*
Stock Number:	006562

Price Details

IMPORTANT NOTE: Prices are based on shipping destination. To view prices, select your shipping destination.

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Supply Details

Standard Supply	Repository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement.
Supply Notes	Usually shipped between four and eight weeks of age. This strain is included in the Mouse Mutant Resource collection.
Licensing	See General Terms and Conditions below
Control Information	View Control Information in Strain Details.

General Terms and Conditions

View JAX^® Mice & Services Conditions of Use.

The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX^® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX^® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX^® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.

Ordering and Purchasing Information

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Contact Information
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Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form

Go to JAX^® Mice Query Form

006561	B6.CBy(Cg)-Gpi1^a-m1J/BrkJ
006560	CBy(Cg)-Gpi1^a-m1J/BrkJ

006564	B6(C)-Kit^W-41J Gusb^mps/BrkJ
006559	B6.C-H2^bm1/ByBir-Gusb^mps/BrkJ
000256	B6.C-H2^bm1/ByBir-Gusb^mps/J
006407	B6.Cg-Gusb^mps/BrkJ
002086	B6.Cg-Gusb^mps Tg(Gus^sx)1Wat/J
005053	NOD.Cg-Prkdc^scid Gusb^mps/SndsJ
001880	STOCK Gusb^mps Tg(GUSB)4Sly/BirJ

005730	129S6.CB(B6)-Del(1)1Brk Gpi1^b/Gpi1^c/BrkMdfJ
002027	129S8/SvEv-Gpi1^c Hprt1^b-m2/J
000600	B6-Gpi1^b x B6CBCa A^w-J/A-T(7;15)9H Gpi1^a/J
000377	B6.C-H22^c Gpi1^a/(HW38)ByJ
000123	B6.C-H24^c Gpi1^a/(HW54)ByJ
001622	B6.CAST-Gpi1^a.Cg-Hba^th-J
000797	B6.CAST-Gpi1^a/EiJ
002074	B6.Cg-Gpi1^a Hbb^d H1^b/DehJ
001317	B6.Cg-Igh^a Thy1^a Gpi1^a/J
000909	B6CBy-Gpi1^a/Gpi1^b/J or Gpi1^a-m1/Gpi1^b/J
002800	C3.Cg-Gpi1^a/J
003585	NOD.B6-(Gpi1-D7Mit346)/LtJ

005643	B6.129X-Gusb^tm1Sly/J
005644	B6.129X-Gusb^tm3Sly/J
006557	B6.C3-Gusb^mps-2J/BrkJ
006558	B6.Cg-H2^bm1 Tg(GUSB)4Sly/SndsJ
003525	C3H/HeOuJ-Gusb^mps-2J/BrkJ
005322	C57BL/6J-Gusb^mps-3J/J
001880	STOCK Gusb^mps Tg(GUSB)4Sly/BirJ

Strain Name:

B6.CBy(Cg)-Gusbmps Gpi1a-m1J/BrkJ

Stock Number:

006562

Availability:

Repository- Live

General Terms and Conditions

Product Information

Strain Details

Gusbmps/Gusbmps

Gene & Allele Details

Control Information

Colony Maintenance

Related Strains

Additional Web Information

Animal Health Reports

Research Applications

References

Price and Supply Information

Price Details

*NO Shipping Destination selected!

Supply Details

General Terms and Conditions

The Jackson Laboratory's Genotype Promise

B6.CBy(Cg)-Gusb^mps Gpi1^a-m1J/BrkJ

Gusb^mps/Gusb^mps