Drug-induced effects on urea cycle enzymes associate with altered ammonia metabolism, NAD + biosynthesis and mitochondrial protein acetylome

2018 ◽  
Vol 120 ◽  
pp. S108
Author(s):  
Marco F Moedas ◽  
Mafalda Farelo ◽  
João Caio ◽  
Antonina Pereira ◽  
Lodewijk IJlst ◽  
...  
1984 ◽  
Vol 246 (5) ◽  
pp. R805-R810 ◽  
Author(s):  
J. W. Campbell ◽  
J. E. Vorhaben ◽  
D. D. Smith

Glutamine synthetase, a mitochondrial enzyme in liver of uricotelic reptiles and birds, is present in the cytosolic compartment of Phyllomedusa sauvagei liver. The average level is sufficient to account for the rate of uric acid excretion by adult frogs but is far lower than that present in birds and reptiles. Except for lower carbamoylphosphate synthetase activity, the activities of the urea cycle enzymes in P. sauvagei liver are comparable with those in adult ureotelic amphibians. The subcellular distribution of the urea cycle enzymes is much the same as in ureotelic amphibians and mammals with the possible exception of the occurrence of a small percentage of the carbamoylphosphate synthetase and ornithine transacarbamylase activities in the cytosol. In keeping with the subcellular localization of the enzymes, citrulline, and not glutamine, is formed by isolated liver mitochondria. The rapid degradation of glutamine by these mitochondria suggests a high degree of compartmentation of glutamine in the cytosol of P. sauvagei if it is to function as a precursor of uric acid in this compartment.


PEDIATRICS ◽  
1975 ◽  
Vol 56 (6) ◽  
pp. 1081-1084
Author(s):  
M. Michael Thaler

The brief history of Reye's syndrome has been one of progressive familiarity with a deepening mystery. It is possible that the syndrome reflects a variety of etiologically unrelated conditions with similar clinicopathological manifestations. In this view, subclinical defects in ammonia metabolism may constitute a distinct, identifiable group among disorders responsible for encephalopathy with fatty liver. Awareness of the possible role of mutant urea cycle enzymes and deficient substrates in the pathogenesis of this important illness should stimulate investigations of enzyme kinetics and intracellular transport mechanisms which may reveal a spectrum of abnonmalities in ammonia metabolism-and a specific therapy for each.


1992 ◽  
Vol 267 (8) ◽  
pp. 5032-5035
Author(s):  
M Horiuchi ◽  
K Kobayashi ◽  
M Tomomura ◽  
M Kuwajima ◽  
Y Imamura ◽  
...  

1974 ◽  
Vol 104 (6) ◽  
pp. 678-683 ◽  
Author(s):  
Winnie Chan ◽  
Marian Wang ◽  
Joel D. Kopple ◽  
Marian E. Swendseid

1995 ◽  
Vol 22 (3) ◽  
pp. 349-355 ◽  
Author(s):  
Niels Tygstrup ◽  
Søren Bak ◽  
Bjørg Krog ◽  
Antonello Pietrangelo ◽  
David A. Shafritz

Sign in / Sign up

Export Citation Format

Share Document