Reaction: Defective DCXR does not reduce L-xylulose to xylitol
- in pathway: Essential pentosuria
Mutations that inactivate DCXR block the conversion of L-xylulose to xylitol. The two mutant alleles that have been characterized by DNA sequencing would encode truncated mRNAs and encode no detectable protein product. One of these mutant alleles, DCXR c.583?C, is annotated here (Pierce et al. 2011; Wang & van Eys 1970).
Reaction - small molecule participants:
H+ [cytosol]
NADPH [cytosol]
L-xylulose [cytosol]
Reactome.org reaction link: R-HSA-5662851
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Reaction input - small molecules:
hydron
NADPH(4-)
L-xylulose
Reaction output - small molecules:
Reactome.org link: R-HSA-5662851