CBA/CaHN-Btk^xid/J

Stock number: 001011
Product name: CBA/N
Research areas: Immunology, Inflammation and Autoimmunity Research, Mouse/Human Gene Homologs, Research Tools

Description

CBA/CaHN-Btk^xid/J mice have a mutation in the Bruton's tyrosine kinase gene (Btk), and are a model of human X-linked immunodeficiency. They have a B-lymphocyte-specific defect that results in an inability to launch an antibody response to thymus-independent type II antigens, although they do produce normal amounts of antibody in response to some protein antigens. They have low serum IgM and IgG3 and a reduced number of B-cells. Moreover, the B-cells that are present have a reduced surface IgM to IgD ratio, which suggests a disorder in B-cell maturation.

Detailed description

CBA/CaHN-Btk^xid/J mice have a mutation in the Bruton's tyrosine kinase gene (Btk), and are a model of human X-linked immunodeficiency. They have a B-lymphocyte-specific defect that results in an inability to launch an antibody response to thymus-independent type II antigens, although they do produce normal amounts of antibody in response to some protein antigens. They have low serum IgM and IgG3 and a reduced number of B-cells. Moreover, the B-cells that are present have a reduced surface IgM to IgD ratio, which suggests a disorder in B-cell maturation.

View Flow Cytometry Characterization Data for Immunodeficient JAX Strains

Development

Btk^xid mice have a mutation in the X-linked Bruton's tyrosine kinase (Btk) gene that was first observed in the CBA/HN colony at the NIH in the early 1970s. Specifically, they contain a C to T substitution at coding nucleotide 82, changing codon 28 from an arginine to a cysteine (p.R28C).

Allele

Symbol: Btk^xid
Name: X linked immune deficiency
MGI accession ID: MGI:1856317
Generation method: Spontaneous
Synonyms: xid
Marker symbol: Btk
Marker name: Bruton agammaglobulinemia tyrosine kinase
Marker synonyms: AGMX1; AT; ATK; BPK; Bruton's tyrosine kinase; IGHD3; IMD1; PSCTK1; xid; XLA; X-linked immune deficiency
Chromosome: X
Strain of origin: CBA/HN
Molecular note: The C to T substitution at coding nucleotide 82 changes codon 28 from an arginine to a cysteine (p.R28C).