B6.Cg-Tg(PRNP-DNAJB2_ia)52aMec/MecH

Status

Available to order

EMMA IDEM:08268
International strain nameB6.Cg-Tg(PRNP-DNAJB2_ia)52aMec/MecH
Alternative nameDNAJB2a overexpressor transgenic line 52
Strain typeTransgenic Strains
Allele/Transgene symbolTg(PRNP-DNAJB2_ia)52aMec,
Gene/Transgene symbolTg(PRNP-DNAJB2_ia)52aMec

Information from provider

ProviderMike Cheetham
Provider affiliationORBIT, UCL Institute of Ophthalmology
Genetic informationThis transgenic overexpressor expresses the short human isoform of DNAJB2 (DNAJB2a) also known as HSJ1a under the control of the bovine PrP promoter to drive neuronal expression. Highest levels of expression are in the nervous system with levels over fold the endogenous mouse DnajB2. This line was shown to reduce polyglutamine protein aggregation in the brain of the R6/2 Huntington's disease model - Labbadia J, Novoselov SS, Bett JS, Weiss A, Paganetti P, Bates, GP, Cheetham ME (2012) Suppression of protein aggregation by chaperone modification of high molecular weight complexes. Brain, 135(4):1180-96, PMID: 22396390.
Phenotypic informationHomozygous:
Mice show no obvious phenotype.

Heterozygous:
Mice show no obvious phenotype. This transgenic overexpressor expresses the short human isoform of DNAJB2 (DNAJB2a), also known as HSJ1a, under the control of the bovine PrP promoter to drive neuronal expression. Highest levels of expression are in the nervous system with levels over fold the endogenous mouse DnajB2. This hemizygous line was shown to reduce polyglutamine protein aggregation in the brain of the R6/2 Huntington's disease model - Labbadia J, Novoselov SS, Bett JS, Weiss A, Paganetti P, Bates, GP, Cheetham ME (2012) Suppression of protein aggregation by chaperone modification of high molecular weight complexes. Brain, 135(4):1180-96, PMID: 22396390. This line has higher expression of hHSJ1a than line 61 (>2-3 fold higher) in the brain and shows a age dependent increase in expression. In contrast line 61 has pretty constant hHSJ1a expression at a lower level in nearly all tissues (although spinal cord level is closer to line 52). This line was used in the Labbadia et al. publication (2012) and line 61 in the Novoselov et al. paper (2013). Line 61 is also available from EMMA (EM:08269).
Breeding historyThe animals were originally from blastocyst injections into B6xCBA cross and were back-crossed onto C57BL/6 for 4 generations before a bottleneck was suspected. A cross to B6xSJL was then made to ensure continued breeding and since then we have backcrossed over 15 generations to C57BL/6. We believe they are now congenic with C57BL/6.
References
  • Suppression of protein aggregation by chaperone modification of high molecular weight complexes.;Labbadia John, Novoselov Sergey S, Bett John S, Weiss Andreas, Paganetti Paolo, Bates Gillian P, Cheetham Michael E, ;2012;Brain : a journal of neurology;135;1180-96; 22396390
Homozygous fertileyes
Homozygous viableyes
Homozygous matings requiredno
Immunocompromisedno

Information from EMMA

Archiving centreMary Lyon Centre at MRC Harwell, Oxford, United Kingdom

Literature references

  • Suppression of protein aggregation by chaperone modification of high molecular weight complexes.;Labbadia John, Novoselov Sergey S, Bett John S, Weiss Andreas, Paganetti Paolo, Bates Gillian P, Cheetham Michael E, ;2012;Brain : a journal of neurology;135;1180-96; 22396390

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