Table 2:

Fibroblast Sialic Acid Levels in Disorders of Free Sialic Acid Metabolism¹

	Fibroblasts whole cell nmol/mg protein (mean ± SD)			Fibroblasts % of free Neu5Ac recovered from
	N	Free Neu5Ac	Bound Neu5Ac	Lysosomal fraction	Soluble fraction	Microsomal fraction	Nuclear fraction
Controls	11	1.0 ± 0.6	14.6 ± 4.6	21 %	54 %	7 %	18 %
Salla Disease2	6	10.0 ± 2.9	11.9 ± 3.7	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

Gray highlights: Abnormal high values compared to controls

¹Extracted from [3, 27, 61]

²Disease nomenclature according to the references from which the data were extracted