A new microtechnique for the analysis of the human hepatic microsomal glucose-6-phosphatase system.
A microtechnique has been developed which enables a complete kinetic analysis of the human hepatic microsomal glucose-6-phosphatase system to be carried out in microsomes isolated from very small liver samples. Complete or partial deficiencies of any of the proteins of the glucose-6-phosphatase system resulting in Type 1a, 1b, 1c or 1d glycogen storage disease can be therefore be diagnosed using hepatic needle biopsy samples, whereas previous methods of diagnosis needed large wedge biopsy samples requiring laparotomy.
Burchell
A
A
Department of Medicine, Ninewells Hospital and Medical School, University of Dundee, Scotland, UK.
Hume
R
R
Burchell
B
B
D005260:Female; D005333:Fetus / Q000201:enzymology; D005952:Glucose-6-Phosphatase / Q000032:analysis*; D006801:Humans; D007231:Infant, Newborn; D008832:Microchemistry / Q000379:methods; D008862:Microsomes, Liver / Q000201:enzymology*; D011247:Pregnancy; D011755:Pyrophosphatases / Q000032:analysis
D005952:Glucose-6-Phosphatase; D011755:Pyrophosphatases