![]() "Mutations in MDH2, Encoding a Krebs Cycle Enzyme, Cause Early-Onset Severe Encephalopathy." Am J Hum Genet 100(1) 151-159. PMID: 8396422ĪitElMkadem17: Ait-El-Mkadem S, Dayem-Quere M, Gusic M, Chaussenot A, Bannwarth S, Francois B, Genin EC, Fragaki K, Volker-Touw CL, Vasnier C, Serre V, van Gassen KL, Lespinasse F, Richter S, Eisenhofer G, Rouzier C, Mochel F, De Saint-Martin A, Abi Warde MT, de Sain-van der Velde MG, Jans JJ, Amiel J, Avsec Z, Mertes C, Haack TB, Strom T, Meitinger T, Bonnen PE, Taylor RW, Gagneur J, van Hasselt PM, Rotig A, Delahodde A, Prokisch H, Fuchs SA, Paquis-Flucklinger V (2017). "Regulation of the cytosolic aspartate aminotransferase housekeeping gene promoter by glucocorticoids, cAMP, and insulin." Biochemistry 32(35) 9065-72. Other References Related to Enzymes, Genes, Subpathways, and Substrates of this PathwayĪggerbeck93: Aggerbeck M, Garlatti M, Feilleux-Duche S, Veyssier C, Daheshia M, Hanoune J, Barouki R (1993). "Biochemistry, 3rd Edition." John Wiley & Sons Inc. "Effect of NAD on Malate Oxidation in Intact Plant Mitochondria." Plant Physiol 66(2) 225-229. Tobin80: Tobin A, Djerdjour B, Journet E, Neuburger M, Douce R (1980). "Citrin and aralar1 are Ca(2+)-stimulated aspartate/glutamate transporters in mitochondria." EMBO J 20(18) 5060-9. Palmieri01: Palmieri L, Pardo B, Lasorsa FM, del Arco A, Kobayashi K, Iijima M, Runswick MJ, Walker JE, Saheki T, Satrustegui J, Palmieri F (2001). "The cytoplasmic malate dehydrogenase in neoplastic tissues presence of a novel isoenzyme?." Br J Cancer 47(5) 727-31. Grisham83: Grisham MB, Bernstein LH, Everse J (1983). "Amino acids and transaminases activity in ventricular CSF and in brain of normal and Alzheimer patients." Neurosci Lett 388(1) 49-53. This pathway also describes aspartate biosynthesis and degradation.ĭAniello05: D'Aniello A, Fisher G, Migliaccio N, Cammisa G, D'Aniello E, Spinelli P (2005). It is quantatively the most important shuttle for the reoxidation of cytosolic NADH in vertebrate tissues under aerobic conditions. The malate-aspartate shuttle yields approximately 3 molecules of ATP per molecule of cytosolic NADH and is found in liver, heart and kidney This shuttle is reversible, so electrons from NADH are brought into the mitochondrion when the NADH/NAD + ratio is higher in the cytosol than in the mitochondrial matrix. In the cytosol, oxaloacetate is regenerated by reversal of the same transamination reaction catalyzed here by Mitochondrial aspartate glutamate transporter 1 simultaneously transports glutamate from the cytosol to the mitochondrial matrix while exporting aspartate from the matrix to the cytosol L-glutamate in a transamination reaction catalyzed byĪspartate aminotransferase 2, and is transformed toĢ-oxoglutarate are transported across the inner mitochondrial membrane. Oxaloacetate cannot cross the mitochondrial membrane. Malate dehydrogenase 2, NAD + is reduced to NADH and a proton is released Malate is then converted back to oxaloacetate by Mitochondrial 2-oxoglutarate/malate transporter simultaneously imports malate into the mitochondrial matrix from the cytosol and exportsĢ-oxoglutarate from the matrix to the cytosol. Mitochondrial 2-oxoglutarate/malate transporter.Ĭytosolic malate dehydrogenase catalyzes the transfer of two electrons from Mitochondrial aspartate glutamate transporter 1 and The shuttle consists of six key component proteins, There are two well established shuttles for the transport of reducing equivalents described for vertebrates, the The shuttle involves a reaction between NADH and an oxidized substrate in the cytosol, the reduced substrate is transported into the mitochondrion and subsequently reoxidized by the electron transfer chain. Malate-aspartate shuttle is a biochemical entity that facilitates the translocation of electrons across the membrane. NADH cannot traverse the inner mitochondrial membrane and cytosolic reducing equivalents (hydrogen atoms) must be transferred to the mitochondria across the membrane via metabolite shuttles that operate between the two compartments NADH is the reducing equivalent that supplies electrons to the electron transport chain to generate ATP. NAD + and the NADH oxidation process in the cell occurs primarily via mitochondrial respiration. NADH must be continuously balanced within the cell to maintain the pathways of anabolism and catabolism. Generation of Precursor Metabolites and Energy Malate/L-aspartate shuttle pathway BioCyc ID: MALATE-ASPARTATE-SHUTTLE-PWY If an enzyme name is shown in bold, there is experimental evidence for this enzymatic activity.
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