Deficiency of the enzyme that converts xylulose to xylitol.
L-gulonic dehydrogenase.
Essential pentosuria.
Biliverdin.
Conversion of glucose 6-phosphate to glucose 1-phosphate via phosphoglucomutase.
It is oxidized at C6 by NAD+-dependent UDP glucose dehydrogenase to form UDP-glucuronic acid.
A hydrogen atom (H+).
It leads to impaired collagen functions, resulting in poor wound healing, osteoporosis, capillary fragility, and anemia.
The HMP Shunt.
It is phagocytosed by macrophages into heme and globin.
Oxidative pathway for glucose metabolism into glucuronic acid, ascorbic acid, and pentoses.
Xylitol dehydrogenase (xylulose reductase).
CO2.
It is conjugated to non-polar acceptor molecules for easier excretion via bile.
3-keto-L-gulonic acid and NADH.
Conversion of Glucose 6-phosphate to Glucose 1-phosphate via phosphoglucomutase.
L-xylulose.
Decarboxylation.
It synthesizes sugar acids for glucuronide synthesis and provides metabolic intermediates.
Scurvy.
Glucose 1-phosphate.
D-xylulose dehydrogenase.
It serves as an alternate oxidative pathway for glucose metabolism into glucuronic acid, ascorbic acid, and pentoses without ATP generation.
Reoxidation of xylitol to D-xylulose.
Deficiencies can lead to clinical disorders arising from deficient enzymes involved in the pathway.
L-gulonic acid.
Glucuronic acid is used in conjugation reactions and is a precursor for ascorbic acid in humans.
By reduction via xylitol dehydrogenase or xylulose reductase.
β-glucuronic acid.
UDP glucuronate.
It is a condition characterized by high quantities of xylulose in urine due to a lack of xylulose reductase.
Formation of L-gulonolactone from L-gulonic acid by lactonase.
It acts as a chain-breaking antioxidant.
L-gulonolactone oxidase.
Vitamin C serves as a cofactor in the synthesis of carnitine from lysine, which is essential for transporting fatty acids to mitochondria.
Reoxidation of xylitol to D-xylulose via D-xylulose dehydrogenase.
Glucose 1-phosphate is activated by UTP via UDP glucose pyrophosphorylase.
Vitamin C acts as a cofactor for dopamine ß-hydroxylase in the conversion of dopamine to norepinephrine.
D-xylulose 5-phosphate.
It oxidizes L-gulonolactone at carbon 2 to form 3-keto-L-gulonolactone.
UDP glucuronic acid.
UDP and D-glucuronic acid.
D-xylulose 5-phosphate.
It enters the hexose monophosphate (HMP) pathway.
By conjugation with D-glucuronate to form diglucuronide.
Dehydroascorbyl radical (As*).
Ascorbate acts as a cofactor to maintain iron in the reduced state (Fe +2) during the hydroxylation of proline and lysine in procollagen.
Essential pentosuria.
Diabetes mellitus.
Humans lack L-gulonolactone oxidase, preventing them from synthesizing ascorbic acid.
It is oxidized to dehydroascorbate during the decarboxylation of α-ketoglutarate.
It serves as a source of glucuronate for reactions involving incorporation into proteoglycans or conjugation with substrates for excretion.
It is used for the synthesis of proteoglycans and for detoxification.
It is involved in the synthesis of proteoglycans and conjugation reactions for excretion of substances like steroid hormones and drugs.
