Fructose 1,6-bisphosphatase deficiency as a cause of childhood interstitial lung disease.

Pediatr Pulmonol

Paediatric Respiratory Medicine, Nottingham Children's Hospital, Nottingham University Hospitals NHS Trust, Queen's Medical Centre, Nottingham, UK.

Published: July 2021

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http://dx.doi.org/10.1002/ppul.25362DOI Listing

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The extreme sensitivity of chicken muscle fructose 1,6-bisphosphatase to inhibition by 5'-AMP has been utilized to develop a new method for the assay of cAMP phosphodiesterase activity. In this method, the substrate (cAMP) is first incubated with phosphodiesterase and the amount of 5'-AMP formed is then determined by measuring the degree of inhibition of fructose 1'6-bisphosphatase activity. The present method conveniently employs the spectrophotometric technique and is sensitive enough to detect the conversion of 50 pmol of cAMP to 5'-AMP in 1 ml of reaction mixture.

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