The erythropoietic response to pyridoxine deprivation in Slc25a38BMKO and p.R411H XLSA male mice. Male mice with the designated genotypes were weaned at age 3 weeks onto a synthetic diet containing defined amounts of pyridoxine. The experiment was discontinued after 3 weeks for Slc25a38BMKO due to the severity of the phenotype. (A-B) Body weight and spleen-to-body weight ratios (A) and selected RBC indices (B) of Slc25a38BMKO mice fed defined pyridoxine diets for 3 weeks. (C) Representative flow cytometry plots of erythroid differentiation determined as CD44 expression as a function of FSC in Ter119+ cells in peripheral blood, BM, and spleen in p.R411H and Slc25a38BMKO animals fed diets containing 10- or 0-ppm pyridoxine. (D) Enumeration of erythroid cell (ter119+) populations in control, p.R411H, and Slc25a38BMKO animals fed diets containing 10- or 0-ppm pyridoxine. †Indicates the unique characteristics of the “transitional population” seen in R411H/Y animals. n = 5 to 7 mutants and n = 9 to 12 control animals per diet. All animals are males, except SLC25A38BMKO mice fed 2 ppm, which are a mix of males and females. The dotted line in each panel indicates the average value in control animals. ∗P < .05; ∗∗P < .01; ∗∗∗P < .001; ∗∗∗∗P < .0001, compared with control animals at the same age. #P < .05; ##P < .01; ###P < .001; ####P < .0001, compared with animals of the same genotype on the 10-ppm pyridoxine control diet. B6, pyridoxine; cRBC, circulating red blood cells; CTL, control; Eblasts, erythroblasts.
Figure 6.

The erythropoietic response to pyridoxine deprivation in Slc25a38BMKO and p.R411H XLSA male mice. Male mice with the designated genotypes were weaned at age 3 weeks onto a synthetic diet containing defined amounts of pyridoxine. The experiment was discontinued after 3 weeks for Slc25a38BMKO due to the severity of the phenotype. (A-B) Body weight and spleen-to-body weight ratios (A) and selected RBC indices (B) of Slc25a38BMKO mice fed defined pyridoxine diets for 3 weeks. (C) Representative flow cytometry plots of erythroid differentiation determined as CD44 expression as a function of FSC in Ter119+ cells in peripheral blood, BM, and spleen in p.R411H and Slc25a38BMKO animals fed diets containing 10- or 0-ppm pyridoxine. (D) Enumeration of erythroid cell (ter119+) populations in control, p.R411H, and Slc25a38BMKO animals fed diets containing 10- or 0-ppm pyridoxine. †Indicates the unique characteristics of the “transitional population” seen in R411H/Y animals. n = 5 to 7 mutants and n = 9 to 12 control animals per diet. All animals are males, except SLC25A38BMKO mice fed 2 ppm, which are a mix of males and females. The dotted line in each panel indicates the average value in control animals. ∗P < .05; ∗∗P < .01; ∗∗∗P < .001; ∗∗∗∗P < .0001, compared with control animals at the same age. #P < .05; ##P < .01; ###P < .001; ####P < .0001, compared with animals of the same genotype on the 10-ppm pyridoxine control diet. B6, pyridoxine; cRBC, circulating red blood cells; CTL, control; Eblasts, erythroblasts.

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