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Triosephosphate isomerase deficiency is a rare autosomal recessive [2] metabolic disorder which was initially described in 1965. [3]It is a unique glycolytic enzymopathy that is characterized by chronic haemolytic anaemia, cardiomyopathy, susceptibility to infections, severe neurological dysfunction, and, in most cases, death in early childhood. [4]
Triose phosphate isomerase is a highly efficient enzyme, performing the reaction billions of times faster than it would occur naturally in solution. The reaction is so efficient that it is said to be catalytically perfect : It is limited only by the rate the substrate can diffuse into and out of the enzyme's active site.
Triosephosphate isomerase is an enzyme that in humans is encoded by the TPI1 gene. This gene encodes an enzyme, consisting of two identical proteins, which catalyzes the isomerization of glyceraldehyde 3-phosphate (G3P) and dihydroxyacetone phosphate (DHAP) in glycolysis and gluconeogenesis .
Triosephosphate isomerase (TPI) is a central enzyme of glycolysis, the main pathway for cells to obtain energy by metabolizing sugars. In humans, certain mutations within this enzyme, which affect the dimerisation of this protein, are causal for a rare disease, triosephosphate isomerase deficiency.
The TIM barrel (triose-phosphate isomerase), also known as an alpha/beta barrel, [1]: 252 is a conserved protein fold consisting of eight alpha helices (α-helices) and eight parallel beta strands (β-strands) that alternate along the peptide backbone. [2] The structure is named after triose-phosphate isomerase, a conserved metabolic enzyme. [3]
Low phosphate levels inhibit GAP dehydrogenase; GAP is instead converted into DHAP by triosephosphate isomerase. Again, increased levels of DHAP activate methylglyoxal synthase and methylglyoxal production (Weber 711–13).
Glucose isomerase (also known as xylose isomerase) catalyzes the conversion of D-xylose and D-glucose to D-xylulose and D-fructose. Like most sugar isomerases, glucose isomerase catalyzes the interconversion of aldoses and ketoses. [24] The conversion of glucose to fructose is a key component of high-fructose corn syrup production.
Triosephosphate isomerase rapidly interconverts dihydroxyacetone phosphate with glyceraldehyde 3-phosphate (GADP) that proceeds further into glycolysis. This is advantageous, as it directs dihydroxyacetone phosphate down the same pathway as glyceraldehyde 3-phosphate, simplifying regulation.