32 The Chemistry of the Injured Cell 



plete glycogen stores in the human liver, but by an unknown 

 mechanism. 



Bilateral removal of the adrenal glands decreases hepatic glyco- 

 gen, with decreased gluconeogenesis it the animal is diabetic. 

 Cortisone increases hepatic glycogen through stimulation of glyco- 

 genesis (Young et al, 1948). The action of epinephrine (adrenaline) 

 in inducing raised blood sugar concentration through glycogenoly- 

 sis has long been known. We discuss the action of insulin later on. 

 The place of sex hormones in carbohydrate metabolism is still un- 

 certain. Thyroid hormone exerts a general effect on metabolism 

 through its influence on the cytochrome mechanism and oxidative 

 phosphorylation in the liver (Maley and Lardy, 1953) . Liver glyco- 

 gen is reduced by giving thyroid extract, partly perhaps as the re- 

 sult of reduction of lactic acid dehydrogenase activity by thyroxin 

 (Vestling and Knoepfelmacher, 1950) . 



GLYCOGEN-STORAGE DISEASE 



Babies are sometimes affected by mysterious diseases in which 

 vast amounts of glycogen are stored within the cells of the liver and 

 kidneys, and on rare occasions in the heart muscle, brain and 

 tongue. The former is often called v. Gierke's disease after the 

 German investigator who described it in 1929. These glycogen 

 storage disorders are not numerically important but they afford a 

 means of studying the structure of glycogen and for this reason they 

 merit close attention. We owe a great debt to Gerty Cori and her 

 collaborators for their brilliant initiation of such studies. 



Five types of glycogen storage are recognised though the evi- 

 dence for some is still scanty (Sant'Agnese, 1959) . The most 

 common type follows the classical pattern established by v. Gierke. 

 The liver and at times the kidneys are primarily affected. They 

 usually are greatly enlarged because they are heavily stocked with 

 glycogen which is normal in structure. But the enzyme glucose-6- 

 phosphatase in the liver, and sometimes in the kidneys, is absent 

 or reduced so that the carbohydrate cycle is interrupted. Glycogeno- 

 lysis does not go on in the usual manner and the child develops 

 hypoglycaemia, ketosis and hyperlipaemia and fails to give a normal 

 glycaemic response after injection of epinephrine. 



