Figure 3: Accumulation of undegraded heparan sulfate occurs in the developing postnatal MPS IIIA mouse brain and cerebral cortex. | Scientific Reports

Figure 3: Accumulation of undegraded heparan sulfate occurs in the developing postnatal MPS IIIA mouse brain and cerebral cortex.

From: Neurodevelopmental Changes in Excitatory Synaptic Structure and Function in the Cerebral Cortex of Sanfilippo Syndrome IIIA Mice

Figure 3

(A) Whole brain glycosaminoglycan uronic acid levels by carbazole assay. (B) Whole brain NRE levels (N-sulfoglucosamine) by LC/MS. (A,B) values are normalized to brain wet weight, which is presented in Fig. S3A. (C) Cerebral cortex heparan sulfate levels measured by LC/MS. (D) Cerebral cortex NRE levels by LC/MS. (E) Sgsh null (Sgsh−/−) cerebral cortex NRE levels by LC/MS. Values normalized to wet tissue mass. Unaffected wild type and heterozygous (Sgsh+/h* open bars) and hypomorphic MPS IIIA (Sgshh/h closed bars), unless otherwise specified. Day of birth = 0 day. N = 3 animals per genotype per age. Transcardial perfusion with PBS was performed on all animals to eliminate contamination from residual blood in tissue. In all cases graphs show mean ± SD.

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