MODULATION OF THE PHENOTYPIC DIVERSITY OF SICKLE-CELL-ANEMIA

Authors
STEINBERG MH
Citation
Mh. Steinberg, MODULATION OF THE PHENOTYPIC DIVERSITY OF SICKLE-CELL-ANEMIA, Hemoglobin, 20(1), 1996, pp. 1-19
Citations number
88
Categorie Soggetti
Biology,Hematology
Journal title
Hemoglobin → ACNP
ISSN journal
03630269
Volume
20
Issue
1
Year of publication
1996
Pages
1 - 19
Database
ISI
SICI code
0363-0269(1996)20:1<1:MOTPDO>2.0.ZU;2-#
Abstract
Sickle cell anemia is noted for being phenotypically heterogeneous. Th is suggests that there are genetic influences that moderate the effect s of the sickle hemoglobin mutation. In this review I focus on genetic ally determined modulation of hemoglobin concentration and fetal hemog lobin levels. Each of these variables has important influences upon si ckle hemoglobin polymerization and the resulting pathophysiology.