DCXR tetramer reduces L-xylulose to xylitol

Stable Identifier
Homo sapiens
L-xylulose + NADPH + H+ <=> xylitol + NADP+
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L-xylulose reductase (DCXR, also known as dicarbonyl/L-xylulose reductase) catalyzes the reversible NADPH dependent reduction of cytosolic L-xylulose to xylitol (Nakagawa et al. 2002). The enzyme is a tetramer (El Kabbani et al.2004) and may be associated with the plasma membrane (Boue et al. 1996). Mutations that inactivate DCXR are associated with essential pentosuria (Pierce et al. 2011; Wang & van Eys 1970).

Literature References
PubMed ID Title Journal Year
4392213 The enzymatic defect in essential pentosuria

Van Eys, J, Wang, YM

N. Engl. J. Med. 1970
8722620 Surface localization of P34H an epididymal protein, during maturation, capacitation, and acrosome reaction of human spermatozoa

Boué, F, Sullivan, R, Blais, J

Biol. Reprod. 1996
22042873 Garrod's fourth inborn error of metabolism solved by the identification of mutations causing pentosuria

King, MC, Fokstuen, S, Lee, MK, Levy-Lahad, E, Motulsky, AG, Maccoss, MJ, Stray, SM, Bereman, MS, Spurrell, CH, Zeligson, S, Pierce, SB, Mandell, JB

Proc. Natl. Acad. Sci. U.S.A. 2011
11882650 Molecular characterization of mammalian dicarbonyl/L-xylulose reductase and its localization in kidney

Asami, J, Hara, A, Tsuritani, K, Kitamura, K, Takahashi, M, Oda, K, Yoshimoto, M, Isaji, T, Otsuka, N, Sakurai, T, Usami, N, Ishikura, S, Nakagawa, J

J. Biol. Chem. 2002
15103634 Crystal structure of human L-xylulose reductase holoenzyme: probing the role of Asn107 with site-directed mutagenesis

Hara, A, Carbone, V, El-Kabbani, O, Darmanin, C, Chung, RP, Usami, N, Ishikura, S

Proteins 2004
Catalyst Activity

L-xylulose reductase (NADP+) activity of DCXR tetramer [plasma membrane]

Orthologous Events
Cross References
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