fructose-1,6-bisphosphatase 1 (D-fructose-1,6- bisphosphate 1-phosphohydrolase 1, FBPase 1, FBP1, FBP)

Function

• carbohydrate biosynthesis, gluconeogenesis
• inhibited by AMP, which affects the turnover of bound substrate & not the affinity for substrate

D-fructose 1,6-bisphosphate + H2O
                <-->
D-fructose 6-phosphate + phosphate

Structure

• homotetramer
• belongs to the FBPase family
• 4 binding sites each for the substrate, for AMP, & for divalent metal cation (the greatest affinity is for Zn+2)

Pathology

• defects in FBP1 are associated with fructose-1,6-bisphosphatase deficiency

More general terms

• fructose-1,6-diphosphatase

Database

• OMIM: https://mirror.omim.org/entry/229700
• UniProt: http://www.uniprot.org/uniprot/P09467.html