TY - JOUR
T1 - Lack of effect of corticosteroids in W/W(v) and Sl/Sl(d) mice
T2 - These strains are not a model for steroid-responsive Diamond-Blackfan anemia
AU - Alter, B. P.
AU - Gaston, T.
AU - Lipton, J. M.
PY - 1993
Y1 - 1993
N2 - W/W(v) and Sl/Sl(d) mice with macrocytic anemias are a potential model for human inherited pure red cell anemia, called Diamond-Blackfan anemia (DBA). The W mutation involves the gene for c-kit, and the Sl mutation the gene for the kit ligand, called mast cell growth factor, steel factor, or stem cell factor. Since many children with DBA respond to treatment with corticosteroids, we administered steroids to these genetically anemic mice, to determine whether they might provide a model for the human disease. There was no improvement in the murine anemia, consistent with other evidence suggesting that mutations in kit or steel may not be involved in Diamond-Blackfan anemia.
AB - W/W(v) and Sl/Sl(d) mice with macrocytic anemias are a potential model for human inherited pure red cell anemia, called Diamond-Blackfan anemia (DBA). The W mutation involves the gene for c-kit, and the Sl mutation the gene for the kit ligand, called mast cell growth factor, steel factor, or stem cell factor. Since many children with DBA respond to treatment with corticosteroids, we administered steroids to these genetically anemic mice, to determine whether they might provide a model for the human disease. There was no improvement in the murine anemia, consistent with other evidence suggesting that mutations in kit or steel may not be involved in Diamond-Blackfan anemia.
KW - corticosteroids
KW - Diamond-Blackfan anemia
KW - mice
UR - http://www.scopus.com/inward/record.url?scp=0027200722&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0027200722&partnerID=8YFLogxK
M3 - Article
C2 - 7686505
AN - SCOPUS:0027200722
SN - 0902-4441
VL - 50
SP - 275
EP - 278
JO - European Journal of Haematology
JF - European Journal of Haematology
IS - 5
ER -