Fig 1: Circulating IHH levels in MPS VII mice were not significantly different from those of normal. Circulating IHH level in normal and MPS VII mice at 14 days, 1 month and 2 months of age. Blood samples were collected from normal and MPS VII mice through cardiac puncture. Results are presented as mean ± S.D. of 5 mice.
Fig 2: IHH production was greatly reduced in MPS VII growth plate. (A) Immunohistochemical staining for IHH on normal and MPS VII mice at P14, 1 month and 2 months of age. (B) Distribution of IHH in the growth plate of P14 mice were presented as percentage of positive stained cells to total cells in each zone. Scale bar = 20 µm. *denotes to significant differences between normal and MPS VII mice (p < 0.05, student's t-test). (C) Level of IHH produced by normal and MPS VII chondrocyte in culture. # denotes significant difference of IHH secretion between normal and MPS VII chondrocytes. (Two-way ANOVA, Turkey post-hoc, p < 0.05). Results were presented as the mean ± S.D. of 3 replicates of pooled chondrocytes.
Fig 3: Exogenous IHH stimulated cell proliferation of MPS VII chondrocytes. (A) Cell proliferation of normal and MPS VII in response to IHH (25 ng/ml) stimulation. Results were presented as the mean ± S.D. of 3 replicates of pooled chondrocytes. * denotes to significant difference of cell proliferation between IHH-treated normal and MPS VII chondrocytes (p < 0.05, student's t-test). (B) Gene expression of IHH receptors patched (Ptch1) and smoothed (Smo) in the PZ of the growth plate, of P14 normal and MPS VII mice. Results were presented as mean ± S.D. of 5 mice.
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