B6.SJL-Tg(CAG-VCP)7Jpat/J

Stock number: 034128
Product name: VCP-wt
Research areas: Cell Biology Research, Internal/Organ Research, Neurobiology Research

Description

VCP-wt transgenic mice express wildtype human VCP (valosin-containing protein) cDNA under the control of the CMV-enhanced chicken beta actin promoter. This strain is useful as a transgenic control for VDP-A232E transgenic mice (Stock No. 034127), which expresses the A232E mutant form of the human VCP gene and develops characteristics of inclusion body myopathy associated with Paget’s disease of bone and frontotemporal dementia (IBMPFD).

Detailed description

Inclusion body myopathy associated with Paget’s disease of bone and frontotemporal dementia (IBMPFD) is a dominantly inherited degenerative disorder caused by mutations in the valosin-containing protein (VCP) gene. To date, a total of 14 VCP gene mutations associated with Paget's disease have been identified. Mutation of alanine 232 to glutamic acid (A232E) is associated with the most severe clinical presentation characterized by an early onset of bone defects and particularly aggressive myopathy.

These VCP-wt transgenic mice express wildtype human VCP/p97 cDNA under the control of the CMV-enhanced chicken beta actin promoter. Western blot analysis of VCP protein levels from muscle, brain, and bone demonstrate a 2-3 fold increase in endogenous levels. This transgene integrated on mouse Chromosome 12 (band 12B). The transgene copy number is unknown.

VCP-wt mice act as a transgenic control for VDP-A232E transgenic mice (Stock No. 034127), which expresses the A232E mutant form of the human VCP gene and develop characteristics of IBMPFD.

Development

Wildtype human VCP/p97 cDNA under the control of the CMV-enhanced chicken beta actin (CAG) promoter was microinjected into male SJL pronuclei, which were then transferred to foster females. Founders carrying the transgene were backcrossed to C57BL/6J for at least 6 generations by the donating laboratory. This is founder line 7. This transgene integrated on mouse Chromosome 12 (band 12B). The transgene copy number is unknown.

Allele

Symbol: Tg(CAG-VCP)7Jpat
Name: transgene insertion 7, J Paul Taylor
MGI accession ID: MGI:5435088
Generation method: Transgenic
Attributes: Inserted expressed sequence
Synonyms: VCP-wt
Marker symbol: Tg(CAG-VCP)7Jpat
Marker name: transgene insertion 7, J Paul Taylor
Chromosome: UN
Strain of origin: SJL
Molecular note: The CAG promoter was used to drive ubiquitous expression of the wild type human cDNA. Four lines were created with line 7 selected as the representative line.