Biology of the Sialic Acids 1995
DOI: 10.1007/978-1-4757-9504-2_10
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Sialic Acid in Biochemical Pathology

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Cited by 7 publications
(5 citation statements)
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“…Certain diseases and pregnancy [39] are characterized by both increased incidence and diversity of sialyloligosaccharides. Salla's disease is a defective lysosomal membrane transport system for SA, in which the level of free SA in urine is about 5-to 10-fold compared with normal [42]. Sewell et al [43] have showed an increased urinary excretion of free SA and confirmed this in cultivated fibroblasts.…”
Section: Urinementioning
confidence: 99%
See 1 more Smart Citation
“…Certain diseases and pregnancy [39] are characterized by both increased incidence and diversity of sialyloligosaccharides. Salla's disease is a defective lysosomal membrane transport system for SA, in which the level of free SA in urine is about 5-to 10-fold compared with normal [42]. Sewell et al [43] have showed an increased urinary excretion of free SA and confirmed this in cultivated fibroblasts.…”
Section: Urinementioning
confidence: 99%
“…In sialuria, a rare defect with excessive synthesis of SA, free ᮊ 1999 Blackwell Science Ltd, European Journal of Clinical Investigation, 29, 413-425 Table 2 Mean Ϯ SD, serum total SA (TSA), lipid-bound SA (LSA), TSA/total protein (TP) and protein-bound SA (PBSA) levels in healthy humans SA levels can be elevated 70-to 200-fold [44,45]. Sialidosis refers to a series of disorders caused by genetic abnormalities in the lysosomal sialidase, which removes the terminal SA from glycoproteins [42]. Several SA-containing glycoproteins originating from plasma are found in urine.…”
Section: Urinementioning
confidence: 99%
“…For the measurement of serum total SA level, Denny's colorimetric method was used (18) . This method is the modified version of Aminoff and Warren methods, which are thiobarbituric acid (TBA) colorimetric assays that are commonly used for the measurement of total SA level (19,20) .…”
Section: Methodsmentioning
confidence: 99%
“…So far, about 150 cases have been reported. 1141,1145 The name "Salla" is derived from a Northern area in Finland from where most of the patients originated. However, two patients with Salla disease were also detected in Turkey.…”
Section: Salla Disease and Infantile Sialic Acid Storage Diseasementioning
confidence: 99%