Fig 1.
Localization of catheter opening and spinal cord slice relation to the vertebrae of the spine.
The tip of the catheter was located at ~T12 or L1. In the procedure of sectioning, a slice at the tip of the catheter was labeled as 0. From this point, the spinal cord was cut in 1 cm sections in both directions. The slices posterior to 0 were negative and the slices anterior to 0 were positive.
Fig 2.
Coronal slicing the monkey brain.
The brain was cut into 16 slices in a brain matrix from frontal to distal direction at 3 mm coronal slice thickness. R, right; L, left.
Table 1.
Basal levels of I2S in selected tissues.
Fig 3.
I2S concentrations in different levels of the spinal cord and in the CSF after IT-L administration.
Note: An outlier in Slice 2 was 23-fold higher than the mean from all other slices and was not included in the mean calculation. The concentration-time data for the CSF is from Xie et al [10]. CM, cisterna magna; CSF, cerebrospinal fluid; I2S, iduronate-2-sulfatase; IT-L, intrathecal-lumbar.
Fig 4.
Comparison of I2S concentrations (ELISA) in the cerebral cortex and the CSF after IT-L administration.
CM, cisterna magna; CSF, cerebrospinal fluid; I2S, iduronate-2-sulfatase; IT-L, intrathecal-lumbar.
Table 2.
Comparison of non-compartmental pharmacokinetic parameters for the cerebral cortex measured by ELISA and 4-MUF (n = 2).
Fig 5.
Comparison of I2S concentrations in the white matter and the CSF after IT-L administration.
CM, cisterna magna; CSF, cerebrospinal fluid; ELISA, enzyme linked immunosorbent assay; I2S, iduronate-2-sulfatase; IT-L, intrathecal-lumbar.
Table 3.
Comparison of non-compartmental pharmacokinetic parameters for the white matter measured by ELISA and 4-MUF (n = 2).
Fig 6.
Correlation and linear regression of I2S concentrations measured by ELISA and bioactivity (4-MUF) assays.
4-MUF, 4-methylumbelliferyl-α-iduronate-2-sulfate; ELISA, enzyme linked immunosorbent assay; I2S, iduronate-2-sulfatase.
Fig 7.
Comparison of I2S concentrations in the kidneys, liver, CSF, and serum after IT-L administration.
ELISA, enzyme linked immunosorbent assay; IT-L, intrathecal-lumbar.
Table 4.
Non-compartmental pharmacokinetic parameters for the liver and kidneys measured by ELISA (n = 2).