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Phenotypes Associated with This Genotype
Genotype
MGI:3764497
Allelic
Composition
Hbatm1Paz/Hbatm1Paz
Hbbtm1Tow/Hbbtm1Tow
Tg(HBA-HBBs)41Paz/?
Genetic
Background
involves: 129S2/SvPas * 129S7/SvEvBrd * Black Swiss * C57BL/6 * DBA/2* FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hbatm1Paz mutation (2 available); any Hba mutation (12 available)
Hbbtm1Tow mutation (1 available); any Hbb mutation (25 available)
Tg(HBA-HBBs)41Paz mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• many pups turn purple and die within a few hours of birth, however, mice that survive live to become adults (reaching at least 7 months of age)

hematopoietic system
• congested sinusoidal channels are observed in spleen
• erythrocytes have increased dynamic rigidity
• average hematocrit levels are 65% of control at 3-7 months of age
• erythrocytes contain an excess of alpha globin chain synthesis
• sickle cell shapes are observed at a frequency of 5-10% in oxygenated blood
• reticulocyte counts are elevated at 3-7 months of age
• weight is increased 13-fold as compared to control
• erythrocytes have decreased osmotic fragility

homeostasis/metabolism
• cause of death in some newborn mice
• iron deposits found in the tubular epithelium of the kidney
• iron deposits found in Kupffer cells of liver

cardiovascular system
• congested sinusoidal channels are observed in spleen
• weight is increased 2-fold as compared to control

renal/urinary system
• iron deposits found in the tubular epithelium of the kidney
• weight is increased 2-fold as compared to control

immune system
• congested sinusoidal channels are observed in spleen
• weight is increased 13-fold as compared to control

liver/biliary system
• iron deposits found in Kupffer cells of liver

respiratory system

Mouse Models of Human Disease
OMIM ID Ref(s)
Sickle Cell Anemia 603903 J:44161


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
04/26/2016
MGI 6.03
The Jackson Laboratory