EC Number   |
General Information |
Reference |
|---|
   2.7.1.28 | evolution |
hTKFC is functionally and structurally similar to Citrobacter sp. DHA kinase |
759363 |
| 2.7.1.28 | evolution |
triose kinase polymorphism demarcates the routes of human migration out of Africa. A nonsynonymous TK allele (rs2260655_A) segregated during human migration out of Africa behaves as TK null for its inability to rescue fructose toxicity and increase hepatic triglyceride accumulation |
759025 |
| 2.7.1.28 | malfunction |
the analyzed TKFC homozygous variants are located within the FMN lyase domain. Functional assays in yeast support the deleterious effect of these variants on protein function. Shared phenotypes between affected individuals with TKFC deficiency include cataracts and developmental delay, associated with cerebellar hypoplasia in one case. Further complications observed in two affected individuals included liver dysfunction and microcytic anemia, while one had fatal cardiomyopathy with lactic acidosis following a febrile illness. Deficiency of TKFC causes disruption of endogenous fructose metabolism leading to generation of by-products that can cause cataract. Deficiency of TKFC leads to impaired innate immunity in response to viral illness, which may explain the fatal illness observed in the most severely affected individual. Phenotypic analyses, oveerview |
758623 |
| 2.7.1.28 | malfunction |
triose kinase deficiency causes oxidative stress and dietary fructose intolerance. In the absence of TK, fructose oxidation is accelerated through the activation of aldehyde dehydrogenase (ALDH) and serine biosynthesis, accompanied by increased oxidative stress and fructose aversion. A nonsynonymous TK allele (rs2260655_A) segregated behaves as TK null for its inability to rescue fructose toxicity and increase hepatic triglyceride accumulation |
759025 |
| 2.7.1.28 | malfunction |
triose kinase deficiency causes oxidative stress and dietary fructose intolerance. In the absence of TK, fructose oxidation is accelerated through the activation of aldehyde dehydrogenase (ALDH) and serine biosynthesis, accompanied by increased oxidative stress and fructose aversion. A nonsynonymous TK allele (rs2260655_A) segregated behaves as TK null for its inability to rescue fructose toxicity and increase hepatic triglyceride accumulation. Deletion of Tk nearly doubles the rate of fructose oxidation, but not the rate of lactate secretion providing an initial indication that TK deficiency may differentially modulate fructose metabolism downstream of glyceraldehyde. Enzyme TK deficiency sensitizes cells to fructose toxicity. TK-deficient mice develop fructose avoidance. TK deficiency reduces hepatic triglyceride accumulation |
-, 759025 |
| 2.7.1.28 | metabolism |
the dual association of fructose with metabolic syndrome and intolerance is controlled by triose kinase (TK) through the metabolic bifurcation of fructose toward oxidative versus lipogenic routes. Fructose is catabolized at a much higher rate than glucose, and triose kinase (TK) couples fructolysis with lipogenesis metabolically and transcriptionally. Triose kinase controls the fate of fructose metabolism glyceraldehyde (GA) represents the only branch point in fructose metabolism |
-, 759025 |
| 2.7.1.28 | more |
study of the flexibility of dimeric hTKFC starting with its active sites in an open conformation, molecular dynamics simulation of the structural model of hTKFC containing the kinase substrates dihydroxyacetone (DHA covalently bound to His221) and ATP. Enzyme structure homology modelling using the crystal structure of Citrobacter sp. DHA kinase. The normal-mode analysis of hTKFC models confirms the trend of K domains to approach L domains, different modes, overview |
759363 |
| 2.7.1.28 | physiological function |
human triokinase/flavin mononucleotide (FMN) cyclase (hTKFC) is a bifunctional enzyme which catalyzes the adenosine triphosphate (ATP)-dependent phosphorylation of D-glyceraldehyde (GA) and dihydroxyacetone (DHA), and also the Mn2+-dependent splitting of flavin adenine dinucleotide (FAD) by an internal cyclizing reaction that forms adenosine monophosphate (AMP) and riboflavin 4',5'-phosphate or cyclic FMN |
759363 |
| 2.7.1.28 | physiological function |
TKFC encodes a bifunctional protein that has been annotated as a homodimeric triokinase and FMN cyclase. Triokinase is a component of the fructose metabolism pathway. Role of TKFC in regulating innate antiviral immunity through suppression of MDA5 |
758623 |
| 2.7.1.28 | physiological function |
triose kinase (TK) constrains fructose oxidation in favor of lipogenic metabolism. Hyperglycemia activates fructolysis through TK to stimulate hepatic lipogenesis. The dual association of fructose with metabolic syndrome and intolerance is controlled by triose kinase (TK) through the metabolic bifurcation of fructose toward oxidative versus lipogenic routes. TK is also required by the endogenous fructolysis pathway to drive lipogenesis and hepatic triglyceride accumulation under high-fat diet and leptin-deficient conditions |
-, 759025 |
