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Science 5 September 1986:
Vol. 233. no. 4768, pp. 1087 - 1089
DOI: 10.1126/science.3090688
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Articles

Science, Vol 233, Issue 4768, 1087-1089
Copyright © 1986 by American Association for the Advancement of Science

articles

Genetic evidence for transmembrane acetylation by lysosomes

KJ Bame and LH Rome

Acetyl-CoA:alpha-glucosaminide N-acetyltransferase is a lysosomal-membrane enzyme deficient in a genetic disorder, Sanfilippo disease type C. The enzyme catalyzes the transfer of an acetyl group from cytoplasmic acetyl-coenzyme A (acetyl-CoA) to terminal alpha-glucosamine residues of heparan sulfate within the organelle. Previous kinetic experiments indicated that the enzyme carries out a transmembrane acetylation via a ping-pong mechanism; the reaction can therefore be dissected into two half reactions--acetylation of the enzyme, and transfer of the acetyl group to glucosamine. Cells derived from patients were found to differ in their ability to perform each half reaction. Five cell lines (derived from three families) were able to catalyze acetylation of the lysosomal membrane and to carry out acetyl-CoA/CoA exchange, whereas a sixth cell line was devoid of this activity.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Localisation of a gene for mucopolysaccharidosis IIIC to the pericentromeric region of chromosome 8.
J Ausseil, J C Loredo-Osti, A Verner, C Darmond-Zwaig, I Maire, B Poorthuis, O P van Diggelen, T J Hudson, T M Fujiwara, K Morgan, et al. (2004)
J. Med. Genet. 41, 941-945
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