B6.Cg-Prkdc^scid/SzJ

Stock number: 001913
Product name: C57BL/6 scid
Strain synonyms: B6 scid
Research areas: Immunology, Inflammation and Autoimmunity Research, Internal/Organ Research, Research Tools, Virology Research

Description

These mice carry the severe combined immune deficiency mutation (scid, which is caused by a spontaneous mutation in the Prkdc gene) on the C57BL/6J strain background. The mutant mice do not have functional T or B cells, thus supporting allogeneic and xenogeneic grafts.

Detailed description

Mice homozygous for the severe combined immune deficiency spontaneous mutation (Prkdc^scid, commonly referred to as scid) are characterized by an absence of functional T cells and B cells, lymphopenia, hypogammaglobulinemia, and a normal hematopoietic microenvironment. Normal antigen-presenting cell, myeloid, and NK cell functions are strain dependent. scid mice carry a DNA repair defect and a defect in the rearrangement of genes that code for antigen-specific receptors on lymphocytes. Most homozygotes have no detectable IgM, IgG1, IgG2a, IgG2b, IgG3, or IgA. Thymus, lymph nodes, and splenic follicles are virtually devoid of lymphocytes. scid mice accept allogeneic and xenogeneic grafts making them an ideal model for cell transfer experiments. Some scid mice will spontaneously develop partial immune reactivity. scid mice that have serum Ig levels greater than 1 ug/ml are considered "leaky." scid leakiness is highly strain dependent, increases with age, and is higher in mice housed under non-SPF conditions. In general, scid leakiness is high on the C57BL/6J and BALB/cBy genetic backgrounds, low on the C3H/HeJ background, and even lower on the NOD/ShiLtSz background.

View Flow Cytometry Characterization Data for Immunodeficient JAX Strains

Development

Prkdc^scid occurred spontaneously in a colony of BALB/c-Igh^b (C.B-17) mice maintained at the Institute for Cancer Research in Philadelphia, PA. The mutation was transferred to the C57BL/6J background by an initial cross between C.B-17-Prkdc^scid/Prkdc^scid and C57BL/6J followed by sequential backcrossing of heterozygous animals of the F1 and male heterozygotes (identified by testcrossing) of subsequent generations to C57BL/6J. At the 10th backcross generation the mutation was made homozygous by brother-sister inbreeding.

Allele

Symbol: Prkdc^scid
Name: severe combined immunodeficiency
MGI accession ID: MGI:1857113
Generation method: Spontaneous
Marker symbol: Prkdc
Marker name: protein kinase, DNA activated, catalytic polypeptide
Chromosome: 16
Strain of origin: C.BKa-Igh^b/Icr
Site of expression: T and B lymphocytes.
Molecular note: A T-to-A transversion point mutation at a position corresponding to codon 4046 (codon 4095 in transcript ENSMUST00000023352.8) created a premature stop codon (p.Y4046*).