| Literature DB >> 176867 |
F A Hommes, K Bendien, J D Elema, H J Bremer, I Lombeck.
Abstract
Two children are described who suffered from hypoglycemia and liver impairment. Assays of gluconeogenic enzymes in liver samples taken immediately after death demonstrated a deficiency of phosphoenolpyruvate carboxykinase, a key enzyme of gluconeogenesis. Post mortem examination demonstrated massive fat deposition in liver and kidney and to a lesser extent in other tissues. The fatty changes in liver and kidney could be explained by the absence of phosphoenolpyruvate carboxykinase, which would cause an alteration in the mitochondrial-cytosolic processes related to gluconeogenesis.Entities:
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Year: 1976 PMID： 176867 DOI： 10.1111/j.1651-2227.1976.tb16543.x
Source DB: PubMed Journal: Acta Paediatr Scand ISSN： 0001-656X