Mitochondrial malic enzyme (E.C. 1.1.1.40) in human leukocytes: Formal genetics and population genetics

1979 ◽  
Vol 51 (3) ◽  
Author(s):  
G. Siebert ◽  
H. Ritter ◽  
J. K�mpf
Keyword(s):  
Population Genetics ◽  
Malic Enzyme ◽  
Human Leukocytes ◽  
Mitochondrial Malic Enzyme

Mitochondrial Malic Enzyme in Human Leukocytes

1987 ◽  
Vol 37 (2) ◽  
pp. 122-123 ◽  
Author(s):  
G. Siebert ◽  
A. Amorim
Keyword(s):  
Malic Enzyme ◽  
Human Leukocytes ◽  
Mitochondrial Malic Enzyme

scholarly journals Regulation of mitochondrial malic enzyme synthesis in mouse brain.

1979 ◽  
Vol 76 (12) ◽  
pp. 6539-6541 ◽  
Author(s):  
E. G. Bernstine ◽  
C. Koh ◽  
C. C. Lovelace
Keyword(s):  
Mouse Brain ◽  
Malic Enzyme ◽  
Enzyme Synthesis ◽  
Mitochondrial Malic Enzyme

scholarly journals Cerebral Metabolism of Lactate in Vivo: Evidence for Neuronal Pyruvate Carboxylation

2000 ◽  
Vol 20 (2) ◽  
pp. 327-336 ◽  
Author(s):  
Bjørnar Hassel ◽  
Anders Bråthe
Keyword(s):  
Malic Enzyme ◽  
Specific Activity ◽  
Cerebral Metabolism ◽  
Resonance Spectroscopy ◽  
Pyruvate Carboxylation ◽  
Nitropropionic Acid ◽  
The Brain ◽  
Mitochondrial Malic Enzyme ◽  
Intrastriatal Injection

The cerebral metabolism of lactate was investigated. Awake mice received [3-13C]lactate or [1-13C]glucose intravenously, and brain and blood extracts were analyzed by 13C nuclear magnetic resonance spectroscopy. The cerebral up-take and metabolism of [3-13C]lactate was 50% that of [1-13C]glucose. [3-13C]Lactate was almost exclusively metabolized by neurons and hardly at all by glia, as revealed by the 13C labeling of glutamate, γ-aminobutyric acid and glutamine. Injection of [3-13C]lactate led to extensive formation of [2-13C]lactate, which was not seen with [1-13C]glucose, nor has it been seen in previous studies with [2-13C]acetate. This formation probably reflected reversible carboxylation of [3-13C]pyruvate to malate and equilibration with fumarate, because inhibition of succinate dehydrogenase with nitropropionic acid did not block it. Of the [3-13C]lactate that reached the brain, 20% underwent this reaction, which probably involved neuronal mitochondrial malic enzyme. The activities of mitochondrial malic enzyme, fumarase, and lactate dehydrogenase were high enough to account for the formation of [2-13C]lactate in neurons. Neuronal pyruvate carboxylation was confirmed by the higher specific activity of glutamate than of glutamine after intrastriatal injection of [1-14C]pyruvate into anesthetized mice. This procedure also demonstrated equilibration of malate, formed through pyruvate carboxylation, with fumarate. The demonstration of neuronal pyruvate carboxylation demands reconsideration of the metabolic interrelationship between neurons and glia.

Population and biochemical genetics of the human mitochondrial malic enzyme

1989 ◽  
Vol 16 (4) ◽  
pp. 369-374 ◽  
Author(s):  
S.S. Papiha ◽  
A. Jackson ◽  
H. Ranasinghe
Keyword(s):  
Malic Enzyme ◽  
Biochemical Genetics ◽  
Mitochondrial Malic Enzyme

scholarly journals Cardiac malic enzyme in Friedreich's disease

1984 ◽  
Vol 11 (S4) ◽  
pp. 643-645 ◽  
Author(s):  
A. Barbeau ◽  
M. Charbonneau ◽  
T. Cloutier ◽  
B. Lemieux
Keyword(s):  
Enzyme Activity ◽  
Enzyme Activities ◽  
Malic Enzyme ◽  
Control Subjects ◽  
Wide Variability ◽  
Malic Enzyme Activity ◽  
Mitochondrial Malic Enzyme

AbstractWe measured the activity of cytosolic and of mitochondrial malic enzyme in the hearts from 4 patients with Friedreich's disease and from two non-ataxic control subjects. There was a wide variability in the results and the slight overall decreases in both enzyme activities were not considered to be statistically significant. From these and other results, we conclude that deficient mitochondrial malic enzyme activity is not a constant or primary feature of Friedreich's disease.

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