Strain Name: |
B6.Cg-Tg(Eno2-Ighmbp2)90Cx Ighmbp2nmd-2J/Cx |
|---|---|
Stock Number: |
003834 |
Availability: | Research Strain |
General Terms and Conditions |
| Former Name |
C57BL/6J-Tg(Eno2-Ighmbp2)90Cx/Cx (Changed: 08-APR-08
) |
|
C57BL/6J-Tg(NSEIghmbp2)90Cx (Changed: 07-APR-08
) | |
|
C57BL/6J-TgN(NSEIghmbp2)90Cx (Changed: 15-DEC-04
) | |
| Genes & Alleles | Ighmbp2; Ighmbp2nmd-2J; Tg(Eno2-Ighmbp2)90Cx; |
Type JAX® GEMM® Strain - Transgenic Additional information on JAX® GEMM® Strains. Mating System Other - see Strain Mating Scheme Text (Female x Male) Tg/0 nmd-2J/+ x +/+ nmd-2J/+ Species laboratory mouse Donating Investigator Gregory Cox, The Jackson Laboratory Strain Description
Mice hemizygous for the transgene are viable and fertile. RT-PCR analysis indicates that transgene expression is limited to the central nervous system including forebrain, cerebellum and spinal cord. The presence of the transgene rescues the neuromuscular degeneration exhibited by nmd-2J mice. These mice have no obvious phenotype. This strain is useful for studies involving the role of Ighmpb2 in motor neuron disease.Strain Development
A transgenic construct was designed with a full-length Ighmbp2 cDNA under the control of the rat enolase 2 promoter. The construct was introduced into C57BL/6J donor eggs. Founder line 90 was consequently established. Transgenic mice were crossed to the spontaneous mutant B6.BKS-Ighmbp2nmd-2J for two generations to generate transgene positive mice homozygous for the nmd-2J.
Mammalian Phenotype Terms assigned by genotype |
| Allele Symbol | Ighmbp2nmd-2J | ||
|---|---|---|---|
| Allele Name | neuromuscular degeneration 2 Jackson | ||
| Common Name(s) | nmd2J; | ||
| Strain of Origin | BKS.Cg-m +/+ Leprdb/J | ||
| Gene Symbol and Name | Ighmbp2, immunoglobulin mu binding protein 2 | ||
| Chromosome | 19 | ||
| Gene Common Name(s) | AEP; CATF1; FLJ34220; FLJ41171; HCSA; HMN6; MGC124598; RIPE3b1; SMARD1; SMUBP2; Smbp-2; Smbp2; immunoglobulin S mu binding protein 2; neuromuscular degeneration; nmd; p110 subunit; sma; spinal muscular atrophy; | ||
| Molecular Note | An A-to-G transition mutation in intron 4 results in the creation of a cryptic splice site and interferes with the normal splicing of the transcript. RT-PCR analysis on RNA derived from various tissues of homozygous mice demonstrated that approximately 20-25% of the transcripts were spliced normally, while 75-80% of the transcripts were spliced aberrantly. [MGI Ref ID J:51890] | ||
| Allele Symbol | Tg(Eno2-Ighmbp2)90Cx | ||
| Allele Name | transgene insertion 90, Gregory A Cox | ||
| Common Name(s) | Tg(Eno2-Ighmpb2)90Cx; | ||
| Molecular Note | A 3.1 kb mouse Ighmbp2 cDNA sequence was placed under the control of a 4 kb sequence containing the rat Eno2 promoter. Transgene expression was limited to the central nervous system as confirmed by RT-PCR expression using primers within exon 1. [MGI Ref ID J:90418] | ||
Strains carrying Ighmbp2nmd-2J allele
002521 B6.BKS-Ighmbp2nmd-2J/J 006514 B6.Cg-Ighmbp2nmd-2J Tg(Ttn-Ighmbp2)108Cx/Cx 006513 B6.Cg-Ighmbp2nmd-2J Tg(Ttn-Ighmbp2)45Cx/Cx 003833 B6.Cg-Tg(Eno2-Ighmpb2)17Cx Ighmbp2nmd-2J/Cx View Strains carrying Ighmbp2nmd-2J (4 strains)
Strains carrying other alleles of Ighmbp2
003833 B6.Cg-Tg(Eno2-Ighmpb2)17Cx Ighmbp2nmd-2J/Cx View Strains carrying other alleles of Ighmbp2 (1 strain)
Ighmbp2nmd-2J related
Mouse/Human Gene Homologs
Spinal Muscular Atrophy with Respiratory Distress Type I (SMARD1)
Neurobiology Research
Neurodegeneration
Neuromuscular Defects
Selected Reference(s)
Additional ReferencesMaddatu TP; Garvey SM; Schroeder DG; Hampton TG; Cox GA. 2004. Transgenic rescue of neurogenic atrophy in the nmd mouse reveals a role for Ighmbp2 in dilated cardiomyopathy. Hum Mol Genet 13(11):1105-15. [PubMed: 15069027] [MGI Ref ID J:90418]
| Strain Name: | B6.Cg-Tg(Eno2-Ighmbp2)90Cx Ighmbp2nmd-2J/Cx |
| Stock Number: | 003834 |
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| Standard Supply | Research Strain. Availability determined by The Jackson Laboratory scientist holding the strain. |
|---|---|
| Licensing | See General Terms and Conditions below |
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