Huizing et al. Page 28 Table 2: 1 A Fibroblast Sialic Acid Levels in Disorders of Free Sialic Acid Metabolism uthor Man Fibroblasts whole cell Fibroblasts nmol/mg protein (mean ± SD) % of free Neu5Ac recovered from N Free Neu5Ac Bound Neu5Ac Lysosomal Soluble fraction Microsomal Nuclear fraction fraction fraction uscr Controls 11 1.0 ± 0.6 14.6 ± 4.6 21 % 54 % 7 % 18 % ipt Salla 2 6 10.0 ± 2.9 11.9 ± 3.7 Disease 66 % 10 % 54 % 5.5 % ISSD2 5 139 ± 92 14.1 ± 10 Sialuria 3 143 ± 35 8.9 ± 11 4 % 88 % 2 % 6 % Abbreviations: ISSD: infantile sialic acid storage disorder; SD: standard deviation A Gray highlights: Abnormal high values compared to controls uthor Man 1Extracted from [3, 27, 61] 2Disease nomenclature according to the references from which the data were extracted uscr ipt A uthor Man uscr ipt A uthor Man uscr ipt Neurosci Lett. Author manuscript; available in PMC 2021 June 11.