Premium
Characterization of human fructose‐1,6‐bisphosphatase in control and deficient tissues
Author(s) -
Adams A.,
Redden C.,
Menahem S.
Publication year - 1990
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01800207
Subject(s) - fructose 1,6 bisphosphatase , glycolysis , gluconeogenesis , phosphofructokinase , biochemistry , biology , in vivo , fructose , enzyme , medicine , genetics
Summary The regulatory properties of human liver and muscle fructose‐1, 6‐bisphosphatases (FBPase) have been studied in control tissues obtained at autopsy and in tissues from a neonate with FBPase deficiency who died as a result of an overwhelming acidosis. Evidence is presented which suggests that the alkaline isoenzyme of FBPase, which is widely regarded as a laboratory artefact, may have an important role in vivo in the regulation and control of glycolysis and gluconeogenesis. FBPase exhibits the hysteretic and dissociative properties associated with regulatory enzymes, and many of the factors which effect FBPase have inverse effects on phosphofructokinase activity, thus providing an integrated regulatory cycle for the control of the direction and rate of flux through the glycolytic pathway.