Figure 1

Expression and localisation of C1q in hSOD1^G93Aand wild-type mice during disease progression. (A) mRNA expression profile of C1qB in lumbar spinal cord of hSOD1^G93A mice relative to wild-type (WT) mice. Dashed line, baseline expression in WT controls at each time point. (B) Degree of immunolabelling for C1q significantly increased in the lumbar spinal cord of hSOD1^G93A mice at end stage when compared with WT mice. (A, B) Data expressed as mean ± standard error of the mean (n = 6 mice/group; *P <0.05, **P <0.01, ***P <0.001, Student t test). (C) to (T) Double immunolabelling of C1q (red) with cellular markers (green) for motor neurons (ChAT; (C) to (E) WT mice, (L) to (N) hSOD1^G93A mice), astrocyte (glial fibrillary acidic protein (GFAP); (F) to (H) WT mice, (O) to (Q) hSOD1^G93A mice), and microglia (Iba-1; (I) to (K) WT mice, (R) to (T) hSOD1^G93A mice) in the ventral lumbar spinal cord of WT and hSOD1^G93A mice (end stage). There was minimal expression of C1q in WT (C, F and I) with marked increase in hSOD1^G93A mice (L, O and R). In hSOD1^G93A mice, C1q was co-localised with ChAT-positive motor neurons (white arrows in (L) and (N) (detailed in U)). There was little to no co-localisation of C1q with GFAP-positive astrocytes (Q (detailed in V)), and minimal co-localisation with Iba-1-labelled microglia (white arrows in R and T (detailed in W)). PS, pre-symptomatic; OS, onset; MS, mid-symptomatic; ES, end-stage. Scale bar for all panels = 20 μm.