Differential labelling of UDP-N-acetylglucosamine in Huntington's-chorea fibroblasts.

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1981-05-15

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Abstract

The hypothesis that there is impaired endogenous synthesis of glucosamine 6-phosphate in Huntington's-chorea fibroblasts was tested by double labelling matched pairs of fibroblasts in culture with carrier-free H3 32PO4 and [U-14C]glucosamine. The [32P]UDP-N-acetyl[14C]glucosamine and [14C]glucosamine 6-[32P]phosphate of the cellular soluble fraction was isolated by charcoal column and paper chromatography. There is no quantitative difference in 32P but a significant difference in 14C in these two sugars in a ratio of approx. 1.5 for Huntington's-chorea fibroblasts compared with normal fibroblasts.

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Scholars@Duke

Tourian

Ara Y. Tourian

Associate Professor Emeritus of Medicine

Metabolic Basis of Inherited Central Nervous Disorders 
Biochemical genetics and metabolism
Key Words:
Phenylalanine Hydroxylase, Phenylketonuria, 
Huntington's Chorea: Membrane abnormalities, Glucosamine metabolism, Cell Death   
Wilson's Disease, Alzheimer's Disease

Chronic central pain secondary to central and peripheral nervous system damage:
Strategies for multidisciplinary evaluation and treatment: Centrally acting non-narcotic medications. 


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