ALDOB

Function

Catalyzes the aldol cleavage of fructose 1,6-biphosphate to form two triosephosphates dihydroxyacetone phosphate and D-glyceraldehyde 3-phosphate in glycolysis as well as the reverse stereospecific aldol addition reaction in gluconeogenesis. In fructolysis, metabolizes fructose 1-phosphate derived from the phosphorylation of dietary fructose by fructokinase into dihydroxyacetone phosphate and D-glyceraldehyde (PubMed:10970798, PubMed:12205126, PubMed:20848650). Acts as an adapter independently of its enzymatic activity, exerts a tumor suppressor role by stabilizing the ternary complex with G6PD and TP53 to inhibit G6PD activity and keep oxidative pentose phosphate metabolism in check (PubMed:35122041).

Involvement in disease

Hereditary fructose intolerance

HFI

Autosomal recessive disease that results in an inability to metabolize fructose and related sugars. Complete exclusion of fructose results in dramatic recovery; however, if not treated properly, HFI subjects suffer episodes of hypoglycemia, general ill condition, and risk of death the remainder of life.

None

The disease is caused by variants affecting the gene represented in this entry.

Pathway

Carbohydrate degradation; glycolysis; D-glyceraldehyde 3-phosphate and glycerone phosphate from D-glucose: step 4/4.

Carbohydrate biosynthesis; gluconeogenesis.

Carbohydrate metabolism; fructose metabolism.

Sequence Similarities

Belongs to the class I fructose-bisphosphate aldolase family.

Cellular localization

• Cytoplasm
• Cytosol
• Cytoplasm
• Cytoskeleton
• Microtubule organizing center
• Centrosome
• Centriolar satellite

Alternative names

ALDB, ALDOB, Fructose-bisphosphate aldolase B, Liver-type aldolase

Database links

swissprot:P05062 swissprot:P09972 omim:612724 entrezGene:230 entrezGene:229 omim:103870