Utilize este identificador para referenciar este registo: http://hdl.handle.net/10400.18/2244
Título: N-acetylglucosamine-1-phosphate transferase, alpha/beta and gamma subunits; N-acetylglucosamine-1- (GNPTAB, GNPTG)
Autor: Coutinho, Maria Francisca
Palavras-chave: Doenças Genéticas
N-acetylglucosamine-1-phosphate transferase
Data: Mai-2014
Editora: Springer-Verlag
Citação: In: Taniguchi N, Honke K, Fukuda M, et al. (eds). Handbook of Glycosyltransferases and Related Genes. Tokyo: Springer-Verlag, 2014
Resumo: 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).
Peer review: yes
URI: http://hdl.handle.net/10400.18/2244
ISSN: 0959-6658
Versão do Editor: http://www.springerreference.com/index/chapterdbid/332158
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