-
1
-
-
0017201717
-
Safranine as a probe of the mitochondrial membrane potential
-
Akerman K.E., and Wikström M.K. Safranine as a probe of the mitochondrial membrane potential. FEBS Lett. 68 (1976) 191-197
-
(1976)
FEBS Lett.
, vol.68
, pp. 191-197
-
-
Akerman, K.E.1
Wikström, M.K.2
-
2
-
-
0035102870
-
Reduction of large neutral amino acid levels in plasma and brain of hyperleucinemic rats
-
Araújo P., Wassermann G.F., Tallini K., Furlanetto V., Vargas C.R., Wannmacher C.M., Dutra-Filho C.S., Wyse A.T., and Wajner M. Reduction of large neutral amino acid levels in plasma and brain of hyperleucinemic rats. Neurochem. Int. 38 (2001) 529-537
-
(2001)
Neurochem. Int.
, vol.38
, pp. 529-537
-
-
Araújo, P.1
Wassermann, G.F.2
Tallini, K.3
Furlanetto, V.4
Vargas, C.R.5
Wannmacher, C.M.6
Dutra-Filho, C.S.7
Wyse, A.T.8
Wajner, M.9
-
3
-
-
0017184389
-
A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding
-
Bradford M.M. A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding. Anal. Biochem. 72 (1976) 248-254
-
(1976)
Anal. Biochem.
, vol.72
, pp. 248-254
-
-
Bradford, M.M.1
-
4
-
-
0042122354
-
Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease
-
Bridi R., Araldi J., Sgarbi M.B., Testa C.G., Durigon K., Wajner M., and Dutra-Filho C.S. Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease. Int. J. Dev. Neurosci. 21 (2003) 327-332
-
(2003)
Int. J. Dev. Neurosci.
, vol.21
, pp. 327-332
-
-
Bridi, R.1
Araldi, J.2
Sgarbi, M.B.3
Testa, C.G.4
Durigon, K.5
Wajner, M.6
Dutra-Filho, C.S.7
-
5
-
-
33646902437
-
Brain pyruvate and 2-oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid
-
Bunik V.I., Raddatz G., Wanders R.J., and Reiser G. Brain pyruvate and 2-oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid. FEBS Lett. 580 (2006) 3551-3557
-
(2006)
FEBS Lett.
, vol.580
, pp. 3551-3557
-
-
Bunik, V.I.1
Raddatz, G.2
Wanders, R.J.3
Reiser, G.4
-
6
-
-
33747773343
-
Cytosolic branched chain aminotransferase (BCATc) mRNA is up-regulated in restricted brain areas of BDNF transgenic mice
-
Castellano S., Macchi F., Scali M., Huang J.Z., and Bozzi Y. Cytosolic branched chain aminotransferase (BCATc) mRNA is up-regulated in restricted brain areas of BDNF transgenic mice. Brain Res. 1108 (2006) 12-18
-
(2006)
Brain Res.
, vol.1108
, pp. 12-18
-
-
Castellano, S.1
Macchi, F.2
Scali, M.3
Huang, J.Z.4
Bozzi, Y.5
-
7
-
-
0002977005
-
Disorders of branched chain amino acid and keto acid metabolism
-
Scriver C.R., Beaudet A.L., Sly W.S., and Valle D. (Eds), McGraw-Hill, New York, New York
-
Chuang D.T., and Shih V.E. Disorders of branched chain amino acid and keto acid metabolism. In: Scriver C.R., Beaudet A.L., Sly W.S., and Valle D. (Eds). The metabolic and molecular basis of inherited disease. 8 (2001), McGraw-Hill, New York, New York 1971-2001
-
(2001)
The metabolic and molecular basis of inherited disease. 8
, pp. 1971-2001
-
-
Chuang, D.T.1
Shih, V.E.2
-
8
-
-
0000299656
-
Disorders of branched chain amino acid and keto acid metabolism
-
Scriver C.R., Beaudet A.L., Sly W.S., and Valle D. (Eds), McGraw-Hill, New York, New York
-
Danner D.J., and Elsas L.J. Disorders of branched chain amino acid and keto acid metabolism. In: Scriver C.R., Beaudet A.L., Sly W.S., and Valle D. (Eds). The metabolic basis of inherited disease. 6 (1989), McGraw-Hill, New York, New York 671-692
-
(1989)
The metabolic basis of inherited disease. 6
, pp. 671-692
-
-
Danner, D.J.1
Elsas, L.J.2
-
9
-
-
0029873904
-
Threshold effects and control of oxidative phosphorylation in nonsynaptic rat brain mitochondria
-
Davey G.P., and Clark J.B. Threshold effects and control of oxidative phosphorylation in nonsynaptic rat brain mitochondria. J. Neurochem. 66 (1996) 1617-1624
-
(1996)
J. Neurochem.
, vol.66
, pp. 1617-1624
-
-
Davey, G.P.1
Clark, J.B.2
-
10
-
-
77957003282
-
Mitochondrial respiratory control and the polarographic measurement of ADP/O ratios
-
Estabrook R.W., and Pullman M.E. (Eds), Academic Press, New York
-
Estabrook R.W. Mitochondrial respiratory control and the polarographic measurement of ADP/O ratios. In: Estabrook R.W., and Pullman M.E. (Eds). Methods in Enzymology (1967), Academic Press, New York 41-47
-
(1967)
Methods in Enzymology
, pp. 41-47
-
-
Estabrook, R.W.1
-
11
-
-
0022350446
-
Differential investigation of the capacity of succinate oxidation in human skeletal muscle
-
Fischer J.C., Ruitenbeek W., Berden J.A., Trijbels J.M., Veerkamp J.H., Stadhouders M., Sengers R.C., and Janssen A.J. Differential investigation of the capacity of succinate oxidation in human skeletal muscle. Clin. Chim. Acta. 153 (1985) 23-36
-
(1985)
Clin. Chim. Acta.
, vol.153
, pp. 23-36
-
-
Fischer, J.C.1
Ruitenbeek, W.2
Berden, J.A.3
Trijbels, J.M.4
Veerkamp, J.H.5
Stadhouders, M.6
Sengers, R.C.7
Janssen, A.J.8
-
12
-
-
0036186148
-
Stimulation of lipid peroxidation in vitro in rat brain by the metabolites accumulating in maple syrup urine disease
-
Fontella F.U., Gasse E., Pulronik V., Wannmacher C.M., Klein A.B., Wajner M., and Dutra-Filho C.S. Stimulation of lipid peroxidation in vitro in rat brain by the metabolites accumulating in maple syrup urine disease. Metab. Brain Dis. 17 (2002) 47-54
-
(2002)
Metab. Brain Dis.
, vol.17
, pp. 47-54
-
-
Fontella, F.U.1
Gasse, E.2
Pulronik, V.3
Wannmacher, C.M.4
Klein, A.B.5
Wajner, M.6
Dutra-Filho, C.S.7
-
13
-
-
23244435445
-
The alpha-ketoglutarate-dehydrogenase complex: a mediator between mitochondria and oxidative stress in neurodegeneration
-
Gibson G.E., Blass J.P., Beal M.F., and Bunik V. The alpha-ketoglutarate-dehydrogenase complex: a mediator between mitochondria and oxidative stress in neurodegeneration. Mol. Neurobiol. 31 (2005) 43-63
-
(2005)
Mol. Neurobiol.
, vol.31
, pp. 43-63
-
-
Gibson, G.E.1
Blass, J.P.2
Beal, M.F.3
Bunik, V.4
-
14
-
-
0027408857
-
Branched chain aminotransferase isoenzymes. Purification and characterization of the rat brain isoenzyme
-
Hall T.R., Wallin R., Reinhart G.D., and Hutson S.M. Branched chain aminotransferase isoenzymes. Purification and characterization of the rat brain isoenzyme. J. Biol. Chem. 268 (1993) 3092-3098
-
(1993)
J. Biol. Chem.
, vol.268
, pp. 3092-3098
-
-
Hall, T.R.1
Wallin, R.2
Reinhart, G.D.3
Hutson, S.M.4
-
15
-
-
0027184507
-
Physiology and pathophysiology of organic acids in cerebrospinal fluid
-
Hoffmann G.F., Meier-Augenstein W., Stockler S., Surtees R., Rating D., and Nyhan W.L. Physiology and pathophysiology of organic acids in cerebrospinal fluid. J. Inherit. Metab. Dis. 16 (1993) 648-669
-
(1993)
J. Inherit. Metab. Dis.
, vol.16
, pp. 648-669
-
-
Hoffmann, G.F.1
Meier-Augenstein, W.2
Stockler, S.3
Surtees, R.4
Rating, D.5
Nyhan, W.L.6
-
16
-
-
0001234745
-
Influence of α-keto acids on the respiration of brain in vitro
-
Howell R.K., and Lee M. Influence of α-keto acids on the respiration of brain in vitro. Proc. Soc. Exp. Biol. Med. 113 (1963) 660-663
-
(1963)
Proc. Soc. Exp. Biol. Med.
, vol.113
, pp. 660-663
-
-
Howell, R.K.1
Lee, M.2
-
17
-
-
0024267856
-
Subcellular distribution of branched-chain aminotransferase activity in rat tissues
-
Hutson S.M. Subcellular distribution of branched-chain aminotransferase activity in rat tissues. J. Nutr. 118 (1988) 1475-1481
-
(1988)
J. Nutr.
, vol.118
, pp. 1475-1481
-
-
Hutson, S.M.1
-
18
-
-
0026664229
-
Identification of mitochondrial branched chain aminotransferase and its isoforms in rat tissues
-
Hutson S.M., Wallin R., and Hall T.R. Identification of mitochondrial branched chain aminotransferase and its isoforms in rat tissues. J. Biol. Chem. 267 (1992) 15681-15686
-
(1992)
J. Biol. Chem.
, vol.267
, pp. 15681-15686
-
-
Hutson, S.M.1
Wallin, R.2
Hall, T.R.3
-
19
-
-
0020622642
-
Inactivation of the 2-ketoglutarate and pyruvate dehydrogenase complexes of beef heart by branched chain keto acids
-
Jackson R.H., and Singer T.P. Inactivation of the 2-ketoglutarate and pyruvate dehydrogenase complexes of beef heart by branched chain keto acids. J. Biol. Chem. 258 (1983) 1857-1865
-
(1983)
J. Biol. Chem.
, vol.258
, pp. 1857-1865
-
-
Jackson, R.H.1
Singer, T.P.2
-
20
-
-
0038268475
-
MR diffusion imaging and MR spectroscopy of maple syrup urine disease during acute metabolic decompensation
-
Jan W., Zimmerman R.A., Wang Z.J., Berry G.T., Kaplan P.B., and Kaye E.M. MR diffusion imaging and MR spectroscopy of maple syrup urine disease during acute metabolic decompensation. Neuroradiology 45 (2003) 393-399
-
(2003)
Neuroradiology
, vol.45
, pp. 393-399
-
-
Jan, W.1
Zimmerman, R.A.2
Wang, Z.J.3
Berry, G.T.4
Kaplan, P.B.5
Kaye, E.M.6
-
21
-
-
0034512868
-
Primary human fibroblasts from a maple syrup urine disease patient undergo apoptosis following exposure to physiological concentrations of branched chain amino acids
-
Jouvet P., Kozma M., and Mehmet H. Primary human fibroblasts from a maple syrup urine disease patient undergo apoptosis following exposure to physiological concentrations of branched chain amino acids. Ann. N. Y. Acad. Sci. 926 (2000) 116-121
-
(2000)
Ann. N. Y. Acad. Sci.
, vol.926
, pp. 116-121
-
-
Jouvet, P.1
Kozma, M.2
Mehmet, H.3
-
22
-
-
0037726805
-
Intrinsic and extrinsic uncoupling of oxidative phosphorylation
-
Kadenbach B. Intrinsic and extrinsic uncoupling of oxidative phosphorylation. Biochim. Biophys. Acta 1604 (2003) 77-94
-
(2003)
Biochim. Biophys. Acta
, vol.1604
, pp. 77-94
-
-
Kadenbach, B.1
-
23
-
-
77957014496
-
Intra- and extramitochondrial malate dehydrogenase from chicken and tuna heart
-
Kitto G.B. Intra- and extramitochondrial malate dehydrogenase from chicken and tuna heart. Methods Enzymol. 13 (1969) 106-116
-
(1969)
Methods Enzymol.
, vol.13
, pp. 106-116
-
-
Kitto, G.B.1
-
24
-
-
39749127916
-
Phytanic acid impairs mitochondrial respiration through protonophoric action
-
Komen J.C., Distelmaier F., Koopman W.J., Wanders R.J., Smeitink J., and Willems P.H. Phytanic acid impairs mitochondrial respiration through protonophoric action. Cell Mol. Life Sci. 64 (2007) 3271-3281
-
(2007)
Cell Mol. Life Sci.
, vol.64
, pp. 3271-3281
-
-
Komen, J.C.1
Distelmaier, F.2
Koopman, W.J.3
Wanders, R.J.4
Smeitink, J.5
Willems, P.H.6
-
25
-
-
0037044737
-
Effect of Bcl-2 overexpression on mitochondrial structure and function
-
Kowaltowski A.J., Cosso R.G., Campos C.B., and Fiskum G. Effect of Bcl-2 overexpression on mitochondrial structure and function. J. Biol. Chem. 277 (2002) 42802-42807
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 42802-42807
-
-
Kowaltowski, A.J.1
Cosso, R.G.2
Campos, C.B.3
Fiskum, G.4
-
26
-
-
0017258183
-
Control of pyruvate and bhydroxybutyrate utilization in rat brain mitochondria and its relevance to phenylketonuria and maple syrup urine disease
-
Land J.M., Mowbray J., and Clark J.B. Control of pyruvate and bhydroxybutyrate utilization in rat brain mitochondria and its relevance to phenylketonuria and maple syrup urine disease. J. Neurochem. 26 (1976) 823-830
-
(1976)
J. Neurochem.
, vol.26
, pp. 823-830
-
-
Land, J.M.1
Mowbray, J.2
Clark, J.B.3
-
27
-
-
29144460733
-
Maple syrup urine disease; an inborn error of the metabolism of valine, leucine, and isoleucine associated with gross mental deficiency
-
Mackenzie D.Y., and Woolf L.I. Maple syrup urine disease; an inborn error of the metabolism of valine, leucine, and isoleucine associated with gross mental deficiency. Br. Med. J. 10 (1959) 90-91
-
(1959)
Br. Med. J.
, vol.10
, pp. 90-91
-
-
Mackenzie, D.Y.1
Woolf, L.I.2
-
28
-
-
0036264313
-
Diagnosis and treatment of maple syrup disease: a study of 36 patients
-
Morton D.H., Strauss K.A., Robinson D.L., Puffenberger E.G., and Kelley R.I. Diagnosis and treatment of maple syrup disease: a study of 36 patients. Pediatrics 109 (2002) 999-1008
-
(2002)
Pediatrics
, vol.109
, pp. 999-1008
-
-
Morton, D.H.1
Strauss, K.A.2
Robinson, D.L.3
Puffenberger, E.G.4
Kelley, R.I.5
-
29
-
-
4544264062
-
Mitochondrial membrane potential and aging
-
Nicholls D.G. Mitochondrial membrane potential and aging. Aging Cell 3 (2004) 35-40
-
(2004)
Aging Cell
, vol.3
, pp. 35-40
-
-
Nicholls, D.G.1
-
30
-
-
0026693733
-
Molecular diagnosis of maple syrup urine disease
-
Nobukuni Y., Mitsubuchi H., Ohta K., Akaboshi I., Indo Y., Endo F., and Matsuda I. Molecular diagnosis of maple syrup urine disease. J. Inherit. Metab. Dis. 15 (1992) 827-833
-
(1992)
J. Inherit. Metab. Dis.
, vol.15
, pp. 827-833
-
-
Nobukuni, Y.1
Mitsubuchi, H.2
Ohta, K.3
Akaboshi, I.4
Indo, Y.5
Endo, F.6
Matsuda, I.7
-
32
-
-
0016302539
-
Inhibition by the branched-chain 2-oxo acids of the 2-oxoglutarate dehydrogenase complex in developing rat and human brain
-
Patel M.S. Inhibition by the branched-chain 2-oxo acids of the 2-oxoglutarate dehydrogenase complex in developing rat and human brain. Biochem. J. 144 (1974) 91-97
-
(1974)
Biochem. J.
, vol.144
, pp. 91-97
-
-
Patel, M.S.1
-
33
-
-
0037403502
-
Creatine kinase activity from rat brain is inhibited by branched-chain amino acids in vitro
-
Pilla C., Cardozo R.F., Dutra-Filho C.S., Wyse A.T., Wajner M., and Wannmacher C.M. Creatine kinase activity from rat brain is inhibited by branched-chain amino acids in vitro. Neurochem. Res. 28 (2003) 675-679
-
(2003)
Neurochem. Res.
, vol.28
, pp. 675-679
-
-
Pilla, C.1
Cardozo, R.F.2
Dutra-Filho, C.S.3
Wyse, A.T.4
Wajner, M.5
Wannmacher, C.M.6
-
34
-
-
77957014141
-
Isocitrate dehydrogenase from bovine heart
-
Lowenstein J.M. (Ed), Academic Press, New York
-
Plaut G.W.E. Isocitrate dehydrogenase from bovine heart. In: Lowenstein J.M. (Ed). Methods in Enzymology (1969), Academic Press, New York 34-42
-
(1969)
Methods in Enzymology
, pp. 34-42
-
-
Plaut, G.W.E.1
-
35
-
-
36949032322
-
Inhibition of brain energy metabolism by the branched-chain amino acids accumulating in maple syrup urine disease
-
Ribeiro C.A., Sgaravatti A.M., Rosa R.B., Schuck P.F., Grando V., Schmidt A.L., Ferreira G.C., Perry M.L., Dutra-Filho C.S., and Wajner M. Inhibition of brain energy metabolism by the branched-chain amino acids accumulating in maple syrup urine disease. Neurochem. Res. 33 (2008) 114-124
-
(2008)
Neurochem. Res.
, vol.33
, pp. 114-124
-
-
Ribeiro, C.A.1
Sgaravatti, A.M.2
Rosa, R.B.3
Schuck, P.F.4
Grando, V.5
Schmidt, A.L.6
Ferreira, G.C.7
Perry, M.L.8
Dutra-Filho, C.S.9
Wajner, M.10
-
36
-
-
0023597602
-
Cerebral ischemia and reperfusion: prevention of brain mitochondrial injury by lidoflazine
-
Rosenthal R.E., Hamud F., Fiskum G., Varghese P.J., and Sharpe S. Cerebral ischemia and reperfusion: prevention of brain mitochondrial injury by lidoflazine. J. Cereb. Blood Flow Metab. 7 (1987) 752-758
-
(1987)
J. Cereb. Blood Flow Metab.
, vol.7
, pp. 752-758
-
-
Rosenthal, R.E.1
Hamud, F.2
Fiskum, G.3
Varghese, P.J.4
Sharpe, S.5
-
37
-
-
0037444769
-
Mitochondrial threshold effects
-
Rossignol R., Faustin B., Rocher C., Malgat M., Mazat J.P., and Letellier T. Mitochondrial threshold effects. Biochem. J. 370 (2003) 751-762
-
(2003)
Biochem. J.
, vol.370
, pp. 751-762
-
-
Rossignol, R.1
Faustin, B.2
Rocher, C.3
Malgat, M.4
Mazat, J.P.5
Letellier, T.6
-
38
-
-
1942425094
-
Dysmyelination in the brain of adolescents and young adults with maple syrup urine disease
-
Schonberger S., Schweiger B., Schwahn B., Schwarz M., and Wendel U. Dysmyelination in the brain of adolescents and young adults with maple syrup urine disease. Mol. Genet. Metab. 82 (2004) 69-75
-
(2004)
Mol. Genet. Metab.
, vol.82
, pp. 69-75
-
-
Schonberger, S.1
Schweiger, B.2
Schwahn, B.3
Schwarz, M.4
Wendel, U.5
-
39
-
-
33646354917
-
Rotenone-like action of the branched-chain phytanic acid induces oxidative stress in mitochondria
-
Schönfeld P., and Reiser G. Rotenone-like action of the branched-chain phytanic acid induces oxidative stress in mitochondria. J. Biol. Chem. 281 (2006) 7136-7142
-
(2006)
J. Biol. Chem.
, vol.281
, pp. 7136-7142
-
-
Schönfeld, P.1
Reiser, G.2
-
40
-
-
70349281392
-
Evidence that the major metabolites accumulating in medium-chain acyl-CoA dehydrogenase deficiency disturb mitochondrial energy homeostasis in rat brain
-
Schuck P.F., Ferreira G., da C., Tonin A.M., Viegas C.M., Busanello E.N., Moura A.P., Zanatta A., Klamt F., and Wajner M. Evidence that the major metabolites accumulating in medium-chain acyl-CoA dehydrogenase deficiency disturb mitochondrial energy homeostasis in rat brain. Brain Res. 1296 (2009) 117-126
-
(2009)
Brain Res.
, vol.1296
, pp. 117-126
-
-
Schuck, P.F.1
Ferreira, G.2
da, C.3
Tonin, A.M.4
Viegas, C.M.5
Busanello, E.N.6
Moura, A.P.7
Zanatta, A.8
Klamt, F.9
Wajner, M.10
-
41
-
-
0022249030
-
So-called thiaminresponsive maple syrup urine disease: 15-year follow-up of the original patient
-
Scriver C.R., Clow C.L., and George H.G. So-called thiaminresponsive maple syrup urine disease: 15-year follow-up of the original patient. J. Pediatr. 107 (1985) 763-765
-
(1985)
J. Pediatr.
, vol.107
, pp. 763-765
-
-
Scriver, C.R.1
Clow, C.L.2
George, H.G.3
-
42
-
-
0242543265
-
Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease
-
Sgaravatti A.M., Rosa R.B., Schuck P.F., Ribeiro C.A., Wannmacher C.M., Wyse A.T., Dutra-Filho C.S., and Wajner M. Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease. Biochim. Biophys. Acta 1639 (2003) 232-238
-
(2003)
Biochim. Biophys. Acta
, vol.1639
, pp. 232-238
-
-
Sgaravatti, A.M.1
Rosa, R.B.2
Schuck, P.F.3
Ribeiro, C.A.4
Wannmacher, C.M.5
Wyse, A.T.6
Dutra-Filho, C.S.7
Wajner, M.8
-
43
-
-
0021042770
-
Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease
-
Shigematsu Y., Kikuchi K., Momoi T., Sudo M., Kikawa Y., Nosaka K., Kuriyama M., Haruki S., Sanada K., Hamano N., and Suzuki Y. Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J. Inherit. Metab. Dis. 6 (1983) 183-189
-
(1983)
J. Inherit. Metab. Dis.
, vol.6
, pp. 183-189
-
-
Shigematsu, Y.1
Kikuchi, K.2
Momoi, T.3
Sudo, M.4
Kikawa, Y.5
Nosaka, K.6
Kuriyama, M.7
Haruki, S.8
Sanada, K.9
Hamano, N.10
Suzuki, Y.11
-
44
-
-
0001760204
-
Maple syrup urine disease, with particular reference to dietotherapy
-
Snyderman S.E., Norton P.M., Roitman E., and Holt Jr. L.E. Maple syrup urine disease, with particular reference to dietotherapy. Pediatrics 34 (1964) 454-472
-
(1964)
Pediatrics
, vol.34
, pp. 454-472
-
-
Snyderman, S.E.1
Norton, P.M.2
Roitman, E.3
Holt Jr., L.E.4
-
45
-
-
77957010982
-
Citrate synthase
-
Srere P.A. Citrate synthase. Methods Enzymol. 13 (1969) 3-11
-
(1969)
Methods Enzymol.
, vol.13
, pp. 3-11
-
-
Srere, P.A.1
-
46
-
-
4444293612
-
Branched-chain amino acids and neurotransmitter metabolism: expression of cytosolic branched-chain aminotransferase (BCATc) in the cerebellum and hippocampus
-
Sweatt A.J., Garcia-Espinosa M.A., Wallin R., and Hutson S.M. Branched-chain amino acids and neurotransmitter metabolism: expression of cytosolic branched-chain aminotransferase (BCATc) in the cerebellum and hippocampus. J. Comp. Neurol. 477 (2004) 360-370
-
(2004)
J. Comp. Neurol.
, vol.477
, pp. 360-370
-
-
Sweatt, A.J.1
Garcia-Espinosa, M.A.2
Wallin, R.3
Hutson, S.M.4
-
47
-
-
0020606605
-
Abnormal protein and lipid compositions of the cerebral myelin of a patient with maple syrup urine disease
-
Taketomi T., Kunishita T., Hara A., and Mizushima S. Abnormal protein and lipid compositions of the cerebral myelin of a patient with maple syrup urine disease. Jpn. J. Exp. Med. 53 (1983) 109-116
-
(1983)
Jpn. J. Exp. Med.
, vol.53
, pp. 109-116
-
-
Taketomi, T.1
Kunishita, T.2
Hara, A.3
Mizushima, S.4
-
48
-
-
0008263755
-
Disorders of branched-chain amino acid and organic acid metabolism
-
Stanbury S.B., Wyngaarden J.B., Fredrickson D.S., Goldstein J.L., and Brown M.S. (Eds), McGraw-Hill, New York
-
Tanaka K., and Rosenberg L.E. Disorders of branched-chain amino acid and organic acid metabolism. In: Stanbury S.B., Wyngaarden J.B., Fredrickson D.S., Goldstein J.L., and Brown M.S. (Eds). The Metabolic Basis of Inherited Disease. 5 (1983), McGraw-Hill, New York 440-473
-
(1983)
The Metabolic Basis of Inherited Disease. 5
, pp. 440-473
-
-
Tanaka, K.1
Rosenberg, L.E.2
-
49
-
-
0026553444
-
Maple syrup urine disease: interrelations between branched-chain, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination
-
Treacy E., Clow C.L., Reade T.R., Chitayat D., Mamer O.A., and Scriver C.R. Maple syrup urine disease: interrelations between branched-chain, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination. J. Inherit. Metab. Dis. 15 (1992) 121-135
-
(1992)
J. Inherit. Metab. Dis.
, vol.15
, pp. 121-135
-
-
Treacy, E.1
Clow, C.L.2
Reade, T.R.3
Chitayat, D.4
Mamer, O.A.5
Scriver, C.R.6
-
50
-
-
0034671429
-
Inhibition of Krebs cycle enzymes by hydrogen peroxide: a key role of α-ketoglutarate dehydrogenase in limiting NADH production under oxidative stress
-
Tretter L., and Adam-Vizi V. Inhibition of Krebs cycle enzymes by hydrogen peroxide: a key role of α-ketoglutarate dehydrogenase in limiting NADH production under oxidative stress. J. Neurosci. 20 (2000) 8972-8979
-
(2000)
J. Neurosci.
, vol.20
, pp. 8972-8979
-
-
Tretter, L.1
Adam-Vizi, V.2
-
51
-
-
4544226082
-
Generation of reactive oxygen species in the reaction catalyzed by alpha-ketoglutarate dehydrogenase
-
Tretter L., and Adam-Vizi V. Generation of reactive oxygen species in the reaction catalyzed by alpha-ketoglutarate dehydrogenase. J. Neurosci. 24 (2004) 7771-7778
-
(2004)
J. Neurosci.
, vol.24
, pp. 7771-7778
-
-
Tretter, L.1
Adam-Vizi, V.2
-
52
-
-
64449086258
-
Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease
-
Zinnanti W.J., Lazovic J., Griffin K., Skvorak K.J., Paul H.S., Homanics G.E., Bewley M.C., Cheng K.C., Lanoue K.F., and Flanagan J.M. Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease. Brain 132 (2009) 903-918
-
(2009)
Brain
, vol.132
, pp. 903-918
-
-
Zinnanti, W.J.1
Lazovic, J.2
Griffin, K.3
Skvorak, K.J.4
Paul, H.S.5
Homanics, G.E.6
Bewley, M.C.7
Cheng, K.C.8
Lanoue, K.F.9
Flanagan, J.M.10
|