AMDHD2 hydrolyses GlcNGc-6-P to GlcN6P and CCA

Stable Identifier
R-HSA-6803789
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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Humans are not able to catalyse the formation of N-glycolylneuraminic acid (Neu5Gc) due to an inactive CMAHP enzyme. Neu5Gc can be obtained from dietary sources and must be degraded to avoid accummulation and resultant chronic inflammation known as xenosialitis (Varki et al. 2011). In the Neu5Gc degradation pathway, the putative N-acetylglucosamine-6-phosphate deacetylase (AMDHD2) is thought to irreversibly hydrolyse N-glycolylglucosamine 6-phosphate (GlcNGc-6-P), resulting in the ubiquitous metabolites glycolate (CCA) and glucosamine 6-phosphate (GlcN6P) (Bergfeld et al. 2012).

Literature References
PubMed ID Title Journal Year
21073341 Biomedical differences between human and nonhuman hominids: potential roles for uniquely human aspects of sialic acid biology

Varki, NM, Strobert, E, Dick, EJ, Benirschke, K, Varki, A

Annu Rev Pathol 2011
22692205 Metabolism of vertebrate amino sugars with N-glycolyl groups: elucidating the intracellular fate of the non-human sialic acid N-glycolylneuraminic acid

Bergfeld, AK, Pearce, OM, Diaz, SL, Pham, T, Varki, A

J. Biol. Chem. 2012
Participants
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Catalyst Activity
Catalyst Activity
Title
N-acetylglucosamine-6-phosphate deacetylase activity of AMDHD2 [cytosol]
Physical Entity
Activity
Orthologous Events
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