Defective glucuronic acid transport from lysosomes of infantile free sialic acid storage disease fibroblasts.

doi:10.1042/bj2680621

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Article

Defective glucuronic acid transport from lysosomes of infantile free sialic acid storage disease fibroblasts.

The Biochemical journal - 15 Jun 1990

Blom H J, Andersson H C, Seppala R, Tietze F, Gahl W A

Abstract excerpt

Separation by h.p.l.c. and pulsed amperometric detection were employed to measure glucuronic acid (GlcUA) and other acidic monosaccharides in fibroblasts from patients with infantile free sialic acid storage disease (ISSD) and Salla disease. These lysosomal storage disorders result from defective...

Topics

Biological Transport
Cells, Cultured
Fibroblasts
Glucuronates
Glucuronic Acid
Humans
Lysosomes
Metabolism, Inborn Errors
Mutation
Neuraminic Acids
Sialic Acids
Sugar Acids

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