Fig 6. Htt elimination disrupts brain iron homeostasis.

(A) Representative photographs of immunohistochemical staining for ferritin light chain (Ft). Left panels: cerebella of 21mo CTL noTM and 18mo cKO TM@3mo; middle panels: thalami of 12mo CTL TM@9mo and 12mo cKO TM@9mo; right panels: striata of 21mo CTL noTM and 18mo cKO TM@3mo. Note that Ft levels are already extremely reduced in the thalamus 3 months after Htt elimination (middle panels), and that Ft expression is practically abolished in the cerebellum in the absence of Htt (left panels). (B) Representative western blots of Tfr and Fpn protein expression in 10 month-old brains from CTL and cKO mice TM-treated at 6 months of age. Antibody against actin was used as internal control for loading. Note that Tfr and Fpn levels are increased in TM-treated cKO compared to controls. (C) Quantification of Tfr and Fpn expression levels. Western blots of total protein extracts from 10 month-old brains CTL (n = 5) and cKO (n = 5) TM-treated at 6 months of age were probed with anti-Tfr or anti-Fpn, stripped and re-probed with anti-actin antibody. Bands intensities were quantitated using Image J. Tfr and Fpn levels were normalized over actin levels. Values represent mean relative to controls ± SD (***P<0.001, Student’s t-test).