MDH1 deficiency is a metabolic disorder of the malate–aspartate shuttle associated with early onset severe encephalopathy
Sign Up to like & getrecommendations! Published in 2019 at "Human Genetics"
DOI: 10.1007/s00439-019-02063-z
Abstract: The reversible oxidation of l-malate to oxaloacetate is catalyzed by NAD(H)-dependent malate dehydrogenase (MDH). MDH plays essential roles in the malate–aspartate shuttle and the tricarboxylic acid cycle. These metabolic processes are important in mitochondrial NADH… read more here.
Keywords: aspartate shuttle; malate; mdh1 deficiency; malate aspartate ... See more keywords
Malate and Aspartate Increase L-Arginine and Nitric Oxide and Attenuate Hypertension.
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DOI: 10.1016/j.celrep.2017.04.071
Abstract: Fumarase catalyzes the interconversion of fumarate and L-malate in the tricarboxylic acid cycle. The Dahl salt-sensitive (SS) rat, a model of salt-sensitive hypertension, exhibits fumarase insufficiencies. To investigate the mechanism mediating the effect of fumarase-related… read more here.
Keywords: malate; arginine; hypertension malate; malate aspartate ... See more keywords
Mechanistic stoichiometric relationship between the rates of neurotransmission and neuronal glucose oxidation: Reevaluation of and alternatives to the pseudo-malate-aspartate shuttle model.
Sign Up to like & getrecommendations! Published in 2022 at "Journal of neurochemistry"
DOI: 10.1111/jnc.15619
Abstract: The ~1:1 stoichiometry between the rates of neuronal glucose oxidation (CMRglc-ox-N ) and glutamate (Glu)/γ-aminobutyric acid (GABA)-glutamine (Gln) neurotransmitter (NT) cycling between neurons and astrocytes (VNTcycle ) has been firmly established. However, the mechanistic basis… read more here.
Keywords: oxidation; pseudo malate; glucose oxidation; neuronal glucose ... See more keywords
Ketogenic Diet Treatment of Defects in the Mitochondrial Malate Aspartate Shuttle and Pyruvate Carrier
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DOI: 10.3390/nu14173605
Abstract: The mitochondrial malate aspartate shuttle system (MAS) maintains the cytosolic NAD+/NADH redox balance, thereby sustaining cytosolic redox-dependent pathways, such as glycolysis and serine biosynthesis. Human disease has been associated with defects in four MAS-proteins (encoded… read more here.
Keywords: mitochondrial malate; aspartate shuttle; pyruvate carrier; treatment ... See more keywords