B6.Cg-Tg(CAG-COL4A3BP)#Jsau/Cnbc
Status | Available to order |
EMMA ID | EM:11912 |
International strain name | B6.Cg-Tg(CAG-COL4A3BP)#Jsau/Cnbc |
Alternative name | B6-hGPBP |
Strain type | Transgenic Strains |
Allele/Transgene symbol | Tg(CAG-COL4A3BP)#Jsau |
Gene/Transgene symbol | Tg(CAG-COL4A3BP)#Jsau |
Information from provider
Provider | Juan Bautista Saus Mas |
Provider affiliation | Bioquímica y Biología Molecular, Universidad de Valencia. Facultad de Medicina. |
Genetic information | B6-hGPBP are transgenic C57BL/6 mice that have undergone genomic insertion of the cDNA for FLAG-tagged human Goodpasture antigen binding protein-1 (GPBP-1), also known as CERT-L/CERT1 (large isoform of ceramide transfer protein) or collagen type IV alpha-3-binding protein (COL4A3BP). |
Phenotypic information | Homozygous:Homozygous B6-hGPBP mice have not been obtained so far.Heterozygous:Heterozygous B6-hGPBP mice (homozygous mice have not been obtained) undergo type IV collagen disorganization and deposit of immunoglobulin A in glomerular basement membrane (Revert et al. 2007. Am J Pathol. 171:1419-30.). B6-hGPBP mice express FLAG-tagged human GPBP-1. GPBP (Goodpasture antigen binding protein) is encoded by the COL4A3BP gene. The COL4A3BP pre-mRNA was shown to undergo alternative exon splicing which eliminates exon 11 and renders two mature mRNAs. One encoding the full primary product, called GPBP (Raya et al. 1999. J Biol Chem. 274: 12642–9) and an alternative polypeptide, called GPBPD26, devoid of 26-residue domain encoded by the exon 11 (Raya et al. 2000. J. Biol Chem. 275: 40392–9). GPBPD26 was further shown to transfer ceramide between reticulum endoplasmic and Golgi apparatus and it was renamed as CERT (Hanada et al. 2003. Nature. 426: 803–9). Further, the mRNA of GPBP was shown to undergo translation from canonical and non-canonical initiation sites, the latter being located at the 5’-untraslated region (5’-UTR), yielding two polypeptides: the primary canonical product (77-kDa GPBP) and previously unrecognized, non-canonical product (91-kDa GPBP). In contrast to GPBPD26/CERT, the 77-kDa GPBP was shown to undergo secretion and this to be enhanced by membrane-bound 91-kDa GPBP (Revert et al. 2008. J Biol Chem. 283:30246-55). B6-hGPBP mice express recombinant human FLAG-tagged GPBP-1, but not GPBP-3, so recombinant GPBP-1 secretion is limited. |
Breeding history | To generate this strain, we produced pCAGGS-FLAG-GPBP-1 by inserting the cDNA encoding FLAG-tagged human GPBP-1 into EcoRI site of pCAGGS expression vector provided by Jun-ichi Miyazaki (Osaka University Medical School, Osaka, Japan). A SnaBI-HindIII fragment of pCAGG-FLAG-hGPBP-1 was subcloned into the pVAX1 vector (Invitrogen, Carlsbad, CA) generating pVAX1-pCAGGS-FLAG-hGPBP-1, that was subsequently digested with NruI and PmeI. The linearized DNA fragment was isolated and used for injection into the male pronucleus of fertilized oocytes obtained from a (C57BL/6 x DBA/2)F1 female mouse, where the fusion of pronuclei had not taken place yet. Afterwards, the resulting oocytes were transferred into the oviduct of a pseudopregnant NMRI female, prepared by mating with a sterile male. We screened the resulting offspring for transgene transmission by PCR analysis of genomic DNA extracted from mouse tails using specific GPBP primers. Transgenic mice (F1, 50% C57BL/6 and 50% DBA/2) were backcrossed with C57BL/6 mice for six generations to acquire C57BL/6 genetic background (F2 to F7, 75 to 99.25% C57BL/6 and 25 to 0.75% DBA/2). |
References |
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Homozygous fertile | not known |
Homozygous viable | not known |
Homozygous matings required | no |
Immunocompromised | no |
Information from EMMA
Archiving centre | CNB-CSIC, Centro Nacional de Biotecnologia, Madrid, Spain |
Animals used for archiving | heterozygous C57BL/6J |
Literature references
- Increased Goodpasture antigen-binding protein expression induces type IV collagen disorganization and deposit of immunoglobulin A in glomerular basement membrane.;Revert Fernando, Merino Ramón, Monteagudo Carlos, Macias Jesús, Peydró Amando, Alcácer Javier, Muniesa Pedro, Marquina Regina, Blanco Mario, Iglesias Marcos, Revert-Ros Francisco, Merino Jesús, Saus Juan, ;2007;The American journal of pathology;171;1419-30; 17916599
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