Tg(HBA-HBBs)41Paz
Transgene Detail

Summary

• Symbol: Tg(HBA-HBBs)41Paz
• Name: transgene insertion 41, Chris Paszty
• MGI ID: MGI:3056733
• Synonyms: miniLCRalpha1^Ggamma^Agammadeltabeta^S, Tg(Hu-miniLCRalpha1^Ggamma^Agammadeltabeta^S)
• Transgene: Tg(HBA-HBBs)41Paz Location: unknown
• Alliance: Tg(HBA-HBBs)41Paz page

Transgene origin

• Strain of Origin: FVB/N

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(HBA-HBBs)41Paz expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  HBA1 (3039)

  Hba-a1	(MGI:96015)
  Hba-a2	(MGI:96016)

• Mutation details: The transgene contains sequences encoding the human proteins HBA1 (hemoglobin, alpha 1), HBG2 (hemoglobin, gamma G, fetal component), HBG1 (hemoglobin, gamma A, fetal component), HBD (hemoglobin, delta), and HBB ^S (hemoglobin, beta, sickle allele), and the locus control region (LCR). The HBB^S allele contains an A to T transversion mutation in the sixth codon of HBB which causes an amino acid change from Glu to Val. The transgene promoter is multiple: from alpha and beta globin human loci. Transgenic mice express human alpha hemoglobin, gamma hemoglobin, and sickle cell hemoglobin. (J:44161)

Disease models

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 1 strain available Cell Lines: 0 lines available

Notes

In conjunction with Hba^tm1Paz and Hbb^tm1Tmt, transgenic mice express exclusively human sickle hemoglobin. These mice do not express mouse Hba and Hbb, but do express human HBA and HBB. Although chronically anemic, most of these mice survive for 2 to 9 months and are fertile. A significant percentage of sickle cell mice do not survive to adulthood. These mice display the major genetic, hematologic and histopathologic features found in humans with sickle cell disease: irreversibly sickled red cells, anemia, and multiorgan pathology.

References

• Original: J:44161 Paszty C, et al., Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease [see comments]. Science. 1997 Oct 31;278(5339):876-8
• All: 101 reference(s)