Fibγ^390-396Aprotects SCA-associated glomerular pathology. Fully chimeric sickle mice were followed for 1 year posttransplant; kidney samples were fixed in formalin after the mice were euthanized, and stained with H&E and PAS reagent. Representative kidney sections of Fib^WT SS mice (left panel), Fibγ^390-396A SS mice (middle panel) and semiquantitative scoring of histologic features (right panel) showing glomerulosclerosis (A), inflammatory cell infiltration (B), ischemic lesions (C), glomerular mesangial thickening (D), glomerular mesangial hypercellularity (E), and glomerular enlargement (F) are reduced in Fibγ^390-396A SS mice compared with the Fib^WT SS mice. Histopathology scores ranged from 0 to 5, where 0 represents normal kidney morphology; 1, changes in <20% of glomerular area; 2, 21% to 40% of the glomerular area; 3, 41% to 60% of the glomerular area; 4, 61% to 80% of glomerular area; and 5, severe changes in almost all glomeruli. Similar number of fully chimeric male and female mice were used in the experimental groups. The bars in the graph indicate the mean ± SEM. Statistical analyses of the histologic scores were done by Mann-Whitney U test; statistical significance between Fib^WT SS (n = 7) and Fibγ^390-396A SS (n = 8) mice is indicated as **P ≤ .01, *P ≤ .05.