From: Assessment of cellular cobalamin metabolism in Gaucher disease
Variable | Healthy human fibroblasts | GD fibroblasts | **p-value | ||||
---|---|---|---|---|---|---|---|
Without HOCbl (n=3) | With HOCbl (n = 3) | Without HOCbl (n = 3)* | With HOCbl (n = 3)* | Group | Cobalamin supplementation | Interaction | |
tHcy (nmol/mg) range | 0.21[0.04–0.45] 0.19–0.34 | 0.17[0.12–0.22] 0.15–0.19 | 0.26[−0.12–0.77] 0.19–0.53 | 0.25[0.10–0.40] 0.19–0.31 | 0.180 | 0.180 | 0.154 |
Cys (nmol/mg) range | 34.2[10.21–53.57] 22.24–39.23 | 36.26[6.80–64.97] 23.99–47.40 | 31.67[−10.99–87.88] 22.82–60.85 | 27.93[−22.75–102.04] 22.53–68.48 | 1.000 | 0.818 | 0.954 |
Met (nmol/mg) range | 27.72[15.90–40.19] 23.33–33.09 | 32.49[20.65–40.67] 26.04–33.45 | 39.73[22.03–55.31] 31.50–44.77 | 38.59[23.84–59.61] 36.62–49.96 | 0.015 | 0.589 | 0.103 |
MMA (nmol/mg) range | 0.01[0.01–0.02] 0.01–0.02 | 0.01[0.01–0.03] 0.01–0.02 | 0.02[−0.01–0.05] 0.01–0.03 | 0.02[− 0.02–0.06] 0.01–0.04 | 0.699 | 0.818 | 0.965 |