aldolase b การใช้
- Aldolase B converts it into glyceraldehyde and dihydroxyacetone phosphate ( DHAP ).
- Genetic mutations leading to defects in aldolase B result in a condition called hereditary fructose intolerance.
- Aldolase B is a homotetrameric enzyme, composed of four subunits with molecular weights of 36 kDa with local 222 symmetry.
- The aldol cleavage of fructose 1, 6-bisphosphate by aldolase b demonstrates the different reaction products, dihydroxyacetone phosphate and glyceraldehyde 3-phosphate.
- Due to the lack of functional aldolase B, organisms with HFI cannot properly process F1P, which leads to an accumulation of F1P in bodily tissues.
- A deficiency of aldolase B results in an accumulation of fructose-1-phosphate, and trapping of phosphate ( fructokinase requires adenosine triphosphate ( ATP ) ).
- In fructolysis, the enzyme fructokinase initially produces fructose 1-phosphate, which is split by aldolase B to produce the trioses dihydroxyacetone phosphate ( DHAP ) and glyceraldehyde.
- In humans, aldolase B is encoded by the " ALDOB " gene located on chromosome 9 . The gene is 14, 500 base pairs long and contains 9 exons.
- Though the mechanism aldolase B regulation is unknown, increased " ALDOB " gene transcription in the liver has been noticed with an increase in dietary carbohydrates and decrease in glucagon concentration.
- Hereditary fructose intolerance, or the presence of fructose in the blood ( fructosemia ), is caused by a deficiency of aldolase B, the second enzyme involved in the metabolism of fructose.