B6;129-C3^tm1Crr Man2a1^tm1Jxm/J

Stock number: 009077
Research areas: Developmental Biology Research, Hematological Research, Immunology, Inflammation and Autoimmunity Research, Internal/Organ Research, Research Tools

Description

These C3 (complement component 3) and Man2a1 (mannosidase 2, alpha 1) compound mutant animals develop an autoimmune disease similar to human systemic lupus erythematosus (SLE). As the mice age, symptoms that begin to appear include a rise in anti-nuclear antibody (ANA) titers, abnormal hematology characterized as dyserythropoietic anemia, and nephritis.

Allele

Symbol: C3^tm1Crr
Name: targeted mutation 1, Michael C Carroll
MGI accession ID: MGI:1861799
Generation method: Targeted
Attributes: Null/Knockout
Synonyms: C^3tm1Crr; C3-; mC3^-
Marker symbol: C3
Marker name: complement component 3
Marker synonyms: acylation stimulating protein; AHUS5; ARMD9; ASP; C3a; C3b; complement factor 3; CPAMD1; HEL-S-62p; Plp
Chromosome: 17
Strain of origin: 129S4/SvJae
Molecular note: Insertion of a PGK-neomycin resistance cassette into an exon of the C3 gene deleted sequences that code for the C-terminal region of the beta chain and the N-terminal region of the alpha chain, including the site for processing the pro-C3 molecule. ELISA testing did not detect C3 protein in serum of homozygous mutant mice. A C3 hemolytic assay did not detect functional C3 activity.

Allele

Symbol: Man2a1^tm1Jxm
Name: targeted mutation 1, Jamey Marth
MGI accession ID: MGI:2158937
Generation method: Targeted
Attributes: Null/Knockout
Synonyms: alphaM-II^delta
Marker symbol: Man2a1
Marker name: mannosidase 2, alpha 1
Marker synonyms: AMan II; GOLIM7; Mana2; Mana-2; MANII; Manno; Map-2
Chromosome: 17
Strain of origin: (129X1/SvJ x 129S1/Sv)F1-Kitl⁺
Molecular note: Homologous recombination inserted a floxed neomycin-thymidine kinase expression cassette downstream of the exon that encodes amino acids 236-258, which is part of the lumenal "stem" domain, and introduced an additional loxP site upstream of the exon. Cre-mediated recombination in ES cells excised the exon, intronic sequences, and the neo-tk sequences, leaving a single loxP site in their place. Functional analyses did not detect enzyme activity in Golgi fractions from tissues from homozygous mutant mice.