Figure 4: Contributions of neurons and astrocytes to HS accumulation in the cerebral cortex.
(A) NRE levels in cell lysates prepared from primary mature cortical neural cultures containing a mixture of neurons and glia isolated from unaffected (Sgsh+/h*) and hypomorphic MPS IIIA (Sgshh/h) mice. N = 3 individual animals per genotype spanning two litters. (B) NRE levels in intracellular preparations from mature primary cortical cultures of enriched neurons or astrocytes from unaffected and hypomorphic MPS IIIA mice. Note that differences are presented using a logarithmic scale. N = 3 individual animals per genotype spanning two litters. Cell preparations of neurons and astrocytes were obtained from different animals. (C) Relative Sgsh transcript expression by quantitative RT-PCR in wild type primary cultures enriched for neurons or astrocytes. N = 3–4 individual animals spanning two litters. (D) Genomic arrangement of conditional Sgsh allele and Cre-recombinase drivers. Table shows the affected cell types and developmental stage when cre recombinase is expressed for each strain. (E) NRE levels in the cerebral cortex from mice at 182 days of age. N = 3 animals per genotype.
