Reactome | UXS1 tetramer decarboxylates UDP-glucuronate to UDP-xylose

UXS1 tetramer decarboxylates UDP-glucuronate to UDP-xylose

Stable Identifier

R-HSA-8863761

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi lumen, Golgi membrane

Synonyms

H(+) + UDP-alpha-D-glucuronate => CO2 + UDP-alpha-D-xylose

ReviewStatus

5/5

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UXS1 tetramer decarboxylates UDP-glucuronate to UDP-xylose

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UDP-glucuronic acid decarboxylase 1 (UXS1) decarboxylates UDP-alpha-D-glucuronate (UDP-GlcA) to form UDP-alpha-D-xylose (UDP-Xyl). The reaction is localized to the Golgi apparatus (inferred from studies of the homologous rat enzyme (Moriarity et al. 2002). UXS1 is associated with NAD+ (Eixelberger et al. 2012; Walsh et al. 2015 ). The active form of the enzyme is a tetramer of four UXS1:NAD+ subunits (Polizzi et al 2013). UDP-xylose is then used to initiate glycosaminoglycan biosynthesis on the core protein of proteoglycans.

Literature References

PubMed ID	Title	Journal	Year
25521717	Man o' war mutation in UDP-α-D-xylose synthase favors the abortive catalytic cycle and uncovers a latent potential for hexamer formation Wood, ZA, Kadirvelraj, R, Polizzi, SJ, Walsh, RM, Howard, WW	Biochemistry	2015
11877387	UDP-glucuronate decarboxylase, a key enzyme in proteoglycan synthesis: cloning, characterization, and localization Hurt, KJ, Schnaar, RL, Resnick, AC, Moriarity, JL, Storm, PB, Snyder, SH, Laroy, W	J. Biol. Chem.	2002
23656592	Human UDP-α-d-xylose synthase forms a catalytically important tetramer that has not been observed in crystal structures Wood, ZA, Le Magueres, P, Criswell, AR, Polizzi, SJ, Walsh, RM	Biochemistry	2013
22810237	Structure and mechanism of human UDP-xylose synthase: evidence for a promoting role of sugar ring distortion in a three-step catalytic conversion of UDP-glucuronic acid Brunsteiner, M, Sykora, S, Oppermann, U, Eixelsberger, T, Nidetzky, B, Kavanagh, KL, Egger, S, Brecker, L	J Biol Chem	2012