Mouse Genome Informatics
cx
    Hbbd3th/Hbbd3th
Tg(HBB-AR-HBA2,-HBB*)58Rub/0
Tg(LCR-HBA2,LCR-HBB)11Cos/0

involves: FVB/N * Swiss Webster
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• fewer than expected mice survive until P10
• 20% of mice die by 5 months of age

hematopoietic system
• under deoxygenated conditions, more than 95% of cells sickle in less than 50 seconds and many exhibit profusion of spicules unlike wild-type cells
• delay times for polymerization of hemoglobin are shorter than in Hbbd3th/Hbbd3th Tg(LCR-HBA2,LCR-HBB)11Cos mice
• compared with Hbbd3th/Hbbd3th Tg(LCR-HBA2,LCR-HBB)11Cos mice
• mice exhibit neonatal anemia
• mice exhibit spleen fibrosis unlike wild-type mice
• mice exhibit spleen congestion unlike wild-type mice

homeostasis/metabolism
• after 7 hours of water deprivation, mice exhibit a reduced capacity to concentrate urine compared with similarly treated wild-type mice

cardiovascular system
• mice exhibit dilation of renal afferent vessels unlike wild-type mice
• two younger mice exhibit congestion in the brain unlike wild-type mice
• mice exhibit kidney glomerular congestion unlike wild-type mice
• mice exhibit lung congestion unlike wild-type mice
• mice exhibit spleen congestion unlike wild-type mice
• mice exhibit intra-alveolar hemorrhage unlike wild-type mice

liver/biliary system
• mice exhibit multifocal ischemic infarcts in the liver with dilation of the central vein and a preserved rim of viable cells around the central vein unlike wild-type mice
• older mice exhibit liver scarring and fibrosis with collapsed areas unlike wild-type mice
• in older mice

nervous system
• two younger mice exhibit congestion in the brain, occasional red neurons, and rare pyknotic neurons unlike wild-type mice
• two younger mice exhibit congestion in the brain unlike wild-type mice
• mice exhibit red neurons unlike in wild-type mice

renal/urinary system
• mice exhibit dilation of renal afferent vessels unlike wild-type mice
• mice exhibit kidney glomerular congestion unlike wild-type mice
• after 7 hours of water deprivation, mice exhibit a reduced capacity to concentrate urine compared with similarly treated wild-type mice
• mice exhibit focal fibrosis in the medulla and the cortical-medullary junction unlike wild-type mice
• one mouse exhibits cortical fibrosis

respiratory system
• mice exhibit lung congestion unlike wild-type mice
• mice exhibit intra-alveolar hemorrhage unlike wild-type mice
• in two older mice with some platelet thrombi

immune system
• mice exhibit spleen fibrosis unlike wild-type mice
• mice exhibit spleen congestion unlike wild-type mice

Mouse Models of Human Disease
OMIM IDRef(s)
Sickle Cell Anemia 603903 J:94190