Dicarbonyl/L-xylulose reductase, also known as carbonyl reductase II, is an

enzyme An enzyme () is a protein that acts as a biological catalyst by accelerating chemical reactions. The molecules upon which enzymes may act are called substrate (chemistry), substrates, and the enzyme converts the substrates into different mol ...

that in human is encoded by the DCXR

gene In biology, the word gene has two meanings. The Mendelian gene is a basic unit of heredity. The molecular gene is a sequence of nucleotides in DNA that is transcribed to produce a functional RNA. There are two types of molecular genes: protei ...

located on

chromosome 17 Chromosome 17 is one of the 23 pairs of chromosomes in humans. People normally have two copies of this chromosome. Chromosome 17 spans more than 84 million base pairs (the building material of DNA) and represents between 2.5 and 3% of the total DN ...

Structure

The DCXR gene encodes a membrane

protein Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residue (biochemistry), residues. Proteins perform a vast array of functions within organisms, including Enzyme catalysis, catalysing metab ...

that is approximately 34 kDa in size and composed of 224 amino acids. The protein is highly expressed in the kidney and localizes to the cytoplasmic membrane.

Function

DCSR catalyzes the reduction of several L-xylylose as well as a number of pentoses, tetroses, trioses, alpha-dicarbonyl compounds. The enzyme is involved in carbohydrate metabolism, glucose metabolism, the uronate cycle and may play a role in the water absorption and cellular osmoregulation in the proximal renal tubules by producing

xylitol Xylitol is a chemical compound with the formula , or HO(CH2)(CHOH)3(CH2)OH; specifically, one particular Stereoisomerism, stereoisomer with that structural formula. It is a colorless or white crystalline solid. It is classified as a polyalcoho ...

. In

enzymology An enzyme () is a protein that acts as a biological catalyst by accelerating chemical reactions. The molecules upon which enzymes may act are called substrate (chemistry), substrates, and the enzyme converts the substrates into different mol ...

, an L-xylulose reductase () is an

that

catalyzes Catalysis () is the increase in rate of a chemical reaction due to an added substance known as a catalyst (). Catalysts are not consumed by the reaction and remain unchanged after it. If the reaction is rapid and the catalyst recycles quick ...

the

chemical reaction A chemical reaction is a process that leads to the chemistry, chemical transformation of one set of chemical substances to another. When chemical reactions occur, the atoms are rearranged and the reaction is accompanied by an Gibbs free energy, ...

:xylitol + NADP⁺

\rightleftharpoons

L-xylulose + NADPH + H⁺ Thus, the two substrates of this enzyme are

and NADP⁺, whereas its 3

products Product may refer to: Business * Product (business), an item that can be offered to a market to satisfy the desire or need of a customer. * Product (project management), a deliverable or set of deliverables that contribute to a business solution ...

are L-xylulose,

NADPH Nicotinamide adenine dinucleotide phosphate, abbreviated NADP or, in older notation, TPN (triphosphopyridine nucleotide), is a cofactor used in anabolic reactions, such as the Calvin cycle and lipid and nucleic acid syntheses, which require N ...

, and H⁺. Image:Xylulose.png,

Xylulose Xylulose is a ketopentose, a monosaccharide containing five carbon atoms, and including a ketone functional group. It has the chemical formula . In nature, it occurs in both the L- and D-enantiomers. 1-Deoxyxylulose is a precursor to terpenes via ...

Image:Xylitol-2D-structure.svg,

Xylitol Xylitol is a chemical compound with the formula , or HO(CH2)(CHOH)3(CH2)OH; specifically, one particular Stereoisomerism, stereoisomer with that structural formula. It is a colorless or white crystalline solid. It is classified as a polyalcoho ...

(Note conversion of

ketone In organic chemistry, a ketone is an organic compound with the structure , where R and R' can be a variety of carbon-containing substituents. Ketones contain a carbonyl group (a carbon-oxygen double bond C=O). The simplest ketone is acetone ( ...

alcohol Alcohol may refer to: Common uses * Alcohol (chemistry), a class of compounds * Ethanol, one of several alcohols, commonly known as alcohol in everyday life ** Alcohol (drug), intoxicant found in alcoholic beverages ** Alcoholic beverage, an alco ...

) This enzyme belongs to the superfamily of short-chain

oxidoreductase In biochemistry, an oxidoreductase is an enzyme that catalyzes the transfer of electrons from one molecule, the reductant, also called the electron donor, to another, the oxidant, also called the electron acceptor. This group of enzymes usually ut ...

s, specifically those acting on the CH-OH group of donor with NAD⁺ or NADP⁺ as acceptor. The

systematic name A systematic name is a name given in a systematic way to one unique group, organism, object or chemical substance, out of a specific population or collection. Systematic names are usually part of a nomenclature. A semisystematic name or semitrivi ...

of this enzyme class is xylitol:NADP⁺ 2-oxidoreductase (L-xylulose-forming).

Clinical significance

A deficiency is responsible for

pentosuria Pentosuria, a component of Garrod’s tetrad, is a condition where the sugar L-xylulose, a pentose, presents in the urine in unusually high concentrations. It was characterized as an inborn error of carbohydrate metabolism in 1908. It is associa ...

. The insufficiency of L-xylulose reductase activity causes an

inborn error of metabolism Inborn errors of metabolism form a large class of genetic diseases involving congenital disorders of enzyme activities. The majority are due to defects of single genes that code for enzymes that facilitate conversion of various substances (substrat ...

disease characterized by excessive urinary excretion of L-xylulose. Over-expression and ectopic expression of the protein may be associated with prostate adenocarcinoma.

References

External links

* EC 1.1.1 NADPH-dependent enzymes Enzymes of known structure {{1.1.1-enzyme-stub