NOR.Cg-Prkdc^scid/JsdJ

Stock number: 022396
Product name: NOR.scid
Research areas: Cancer Research, Diabetes and Obesity Research, Immunology, Inflammation and Autoimmunity Research, Internal/Organ Research, Research Tools, Virology Research

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. Prkdc^scid mice accept allogeneic grafts making them an ideal model for cell transfer experiments.

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 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.

Donating investigator indicates this strain may be useful for studying autoimmune diabetes in adoptive transfer settings. This strain will not serve as a host for human xenografts since it carries a variant of Sirpa that does not support human engraftment.

In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. It should be noted that the phenotype of these congenic mice could vary from that originally described. We may modify the strain description if necessary as published results become available.

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 Prkdc^scid mutation was backcrossed onto the NOD/ShiLtSz background as follows: an NOD/ShiLtSz female was bred to a C.B-17-Prkdc^scid male; male Prkdc^scid/+ offspring of the F1/N1 and subsequent generations were mated to NOD/ShiLt females for a total of 10 crosses to NOD/ShiLtSz; at generation N10, Prkdc^scid was made homozygous by brother-sister inbreeding. These mice on a NOD/ShiLT background are available as Stock No. 001303. Subsequently, Dr. Jayne Danska at the Hospital for Sick Children backcrossed these mice to NOR/Jsd inbred mice for 11 generations. These mice were inbred to establish a homozygous colony.

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*).