Townes model mice (B6;129-Hbbtm2(HBG1,HBB*)Tow/Hbbtm3(HBG1,HBB)Tow Hbatm1(HBA)Tow/J, 013071) develop sickle cell disease in the presence of human knock-in alleles that replace mouse α-globin and β-globin. The Berkeley model (STOCK Hbatm1Paz Hbbtm1Tow Tg(HBA-HBBs)41Paz/J, 003342) also develops sickle cell disease due to a human hemoglobin transgene and deficiency in mouse α-globin and β-globin. Aspects of sickle cell disease include:
Body weights and complete hematology data are available in the Mouse Phenome Database.
Table 1: Townes Model Hematology. Submental blood was collected into EDTA-coated microtainers and analyzed using a Siemens Advia 2120 hematology analyzer. Data represent mean and one standard deviation of 10 mice per sex and genotype, aged 8 weeks. Each parameter was analyzed separately by sex using a one-way ANOVA; an * indicates that the sickle genotype was significantly different (P < 0.0001) from both control genotypes. Each column header indicates the human beta-globin genotype and the JAX genotype ordering code.
Townes Model Females | Townes Model Males | |||||
---|---|---|---|---|---|---|
Sickle (S/S; M21) |
Control (A/A; M22) |
Corrected (S/A; M23) |
Sickle (S/S; M21) |
Control (A/A; M22) |
Corrected (A/S; M23) |
|
White blood cells (103/μL) | 37.9 ± 9.1* | 13.1 ± 2.8 | 16.4 ± 3.5 | 29.7 ± 5.9* | 11.9 ± 3.0 | 12.9 ± 2.8 |
Red blood cells (106/μL) | 8.2 ± 0.7* | 13.5 ± 0.5 | 13.5 ± 0.3 | 8.3 ± 1.0* | 13.8 ± 0.7 | 13.7 ± 0.5 |
Hemoglobin (g/dL) | 7.8 ± 0.6* | 12.9 ± 0.5 | 12.6 ± 0.4 | 8.3 ± 1.0* | 13.2 ± 0.8 | 13.0 ± 0.6 |
Reticulocytes (%) | 49.6 ± 4.3* | 6.2 ± 1.3 | 8.0 ± 1.9 | 48.7 ± 2.7* | 6.9 ± 3.4 | 6.7 ± 0.7 |
Reticulocytes (109/L) | 4071 ± 444* | 827 ± 170 | 1084 ± 272 | 4026 ± 485* | 931 ± 411 | 925 ± 113 |
Hematocrit (%) | 36.9 ± 2.7* | 46.0 ± 1.4 | 44.6 ± 1.4 | 38.6 ± 4.5* | 47.9 ± 2.1 | 46.0 ± 1.9 |
Mean Cell Volume (fL) | 45.0 ± 2.0* | 34.1 ± 0.5 | 32.9 ± 0.5 | 46.7 ± 0.7* | 34.8 ± 1.0 | 33.5 ± 0.6 |
Red Cell Distribution Width (%) | 32.3 ± 3.8 | 31.7 ± 1.5 | 34.8 ± 2.4 | 30.3 ± 2.3 | 31.4 ± 3.3 | 32.0 ± 1.6 |
Mean Corpuscular Hemoglobin (pg) | 9.5 ± 0.5 | 9.6 ± 0.2 | 9.3 ± 0.3 | 10.1 ± 0.3 | 9.6 ± 0.4 | 9.5 ± 0.2 |
Platelets (103/μL) | 791 ± 191 | 919 ± 152 | 1068 ± 197 | 614 ± 169 | 1079 ± 137 | 1194 ± 198 |
Table 2: Berkeley Model Hematology. Blood was collected as described in Table 1 from 8-12 mice per sex and genotype. Each parameter was analyzed separately by sex using an unpaired two-tailed t-test; an * indicates that the sickle genotype was significantly different (P < 0.0001) from the control genotype.
Berkeley Model Females | Berkeley Model Males | |||
---|---|---|---|---|
Sickle (Hom Hom Hemi) |
Control (Hom Het Hemi) |
Sickle (Hom Hom Hemi) |
Control (Hom Het Hemi) |
|
White blood cells (103/μL) | 20.0 ± 5.2* | 7.1 ± 2.5 | 25.1 ± 7.1* | 12.3 ± 1.5 |
Red blood cells (106/μL) | 8.2 ± 0.5* | 12.1 ± 0.8 | 7.7 ± 0.5* | 12.8 ± 0.3 |
Hemoglobin (g/dL) | 7.0 ± 0.5* | 12.1 ± 0.8 | 6.4 ± 0.5* | 12.3 ± 0.4 |
Reticulocytes (%) | 36.0 ± 2.7* | 6.0 ± 2.2 | 40.7 ± 1.5* | 7.9 ± 0.8 |
Reticulocytes (109/L) | 2946 ± 254* | 738 ± 294 | 3124 ± 213* | 1010 ± 95 |
Hematocrit (%) | 31.1 ± 2.0* | 39.0 ± 2.3 | 30.0 ± 2.0* | 40.2 ± 1.0 |
Mean Cell Volume (fL) | 37.9 ± 1.0* | 32.4 ± 0.9 | 39.0 ± 1.1* | 31.4 ± 0.7 |
Red Cell Distribution Width (%) | 33.2 ± 1.4 | 31.2 ± 2.0 | 33.6 ± 1.4 | 33.5 ± 1.1 |
Mean Corpuscular Hemoglobin (pg) | 8.5 ± 0.2* | 10.1 ± 0.3 | 8.3 ± 0.2* | 9.6 ± 0.3 |
Platelets (103/μL) | 695 ± 56 | 576 ± 130 | 598 ± 273* | 1162 ± 164 |
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