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N-acetylglucosamine-1-phosphate transferase, alpha/beta and gamma subunits; N-acetylglucosamine-1- (GNPTAB, GNPTG)
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Resumo(s)
GlcNAc-1-phosphotransferase catalyzes the transfer of a GlcNAc-1-phosphate
residue from UDP-GlcNAc to C6 positions of selected mannoses in highmannose-
type oligosaccharides of the hydrolases (Goldberg and Kornfeld
1981; Natowicz et al. 1982; Varki and Kornfeld 1983). At a biological level
this reaction is followed by the removal of the terminal GlcNAc by an
N-acetylglucosamine-1-phosphodiester α-N-acetyl-glucosaminidase, usually
referred to as “uncovering enzyme” (UCE; see Chap. 78; Article ID: 332135).
Sequential action of these two enzymes results in the formation of the
mannose-6-phosphate (Man-6-P) marker, a specific tag acquired by lysosomal
hydrolases that ensures recognition by M6P receptors and delivery to the
endosomal/lysosomal system (Braulke and Bonifacino 2009).
Descrição
Palavras-chave
Doenças Genéticas N-acetylglucosamine-1-phosphate transferase
Contexto Educativo
Citação
In: Taniguchi N, Honke K, Fukuda M, et al. (eds). Handbook of Glycosyltransferases and Related Genes. Tokyo: Springer-Verlag, 2014