-
2
-
-
0001106274
-
Disorders of branched chain amino acid and keto acid metabolism
-
Scriver CR, Beaudet AL, Sly WS, Valle D (eds) McGraw Hill, New York
-
Chuang DT, Shih VE 1995 Disorders of branched chain amino acid and keto acid metabolism. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The Metabolic and Molecular Bases of Inherited Disease. McGraw Hill, New York, pp 1239-1277
-
(1995)
The Metabolic and Molecular Bases of Inherited Disease
, pp. 1239-1277
-
-
Chuang, D.T.1
Shih, V.E.2
-
3
-
-
0010757820
-
Maple syrup urine disease: Branched-chain keto-aciduria
-
Dancis J, Levitz M, Westall RG 1960 Maple syrup urine disease: branched-chain keto-aciduria. Pediatrics 25:72-79
-
(1960)
Pediatrics
, vol.25
, pp. 72-79
-
-
Dancis, J.1
Levitz, M.2
Westall, R.G.3
-
5
-
-
0014869286
-
The configuration of the alloisoleucine present in maple syrup urine disease plasma
-
Halpern B, Pollock GE 1970 The configuration of the alloisoleucine present in maple syrup urine disease plasma. Biochem Med 4:352-356
-
(1970)
Biochem Med
, vol.4
, pp. 352-356
-
-
Halpern, B.1
Pollock, G.E.2
-
6
-
-
0017029194
-
Investigation of the metabolic pattern in maple syrup urine disease by means of glass capillary gas chromatography and mass spectrometry
-
Jakobs C, Solem E, Ek J, Halvorsen K, Jellum E 1977 Investigation of the metabolic pattern in maple syrup urine disease by means of glass capillary gas chromatography and mass spectrometry. J Chromatogr 143:31-38
-
(1977)
J Chromatogr
, vol.143
, pp. 31-38
-
-
Jakobs, C.1
Solem, E.2
Ek, J.3
Halvorsen, K.4
Jellum, E.5
-
7
-
-
0018954107
-
Origin of the two peaks for 2-keto-3-methylvaleric acid produced by the oxidation of the keto acids occurring in maple syrup urine disease
-
Mamer OA, Montgomery JA, Taguchi VY 1980 Origin of the two peaks for 2-keto-3-methylvaleric acid produced by the oxidation of the keto acids occurring in maple syrup urine disease. J Chromatogr 182:221-225
-
(1980)
J Chromatogr
, vol.182
, pp. 221-225
-
-
Mamer, O.A.1
Montgomery, J.A.2
Taguchi, V.Y.3
-
8
-
-
0030581167
-
Determination of R-and S-3-methyl-2-oxopentanoate enantiomers in human plasma: Suitable method for label enrichment analysis
-
Schadewaldt P, Wendel U, Hammen H-W 1996 Determination of R-and S-3-methyl-2-oxopentanoate enantiomers in human plasma: suitable method for label enrichment analysis. J Chromatogr B 682:209-218
-
(1996)
J Chromatogr B
, vol.682
, pp. 209-218
-
-
Schadewaldt, P.1
Wendel, U.2
Hammen, H.-W.3
-
9
-
-
0017085714
-
Demonstration of a new mammalian isoleucine catabolic pathway yielding an R series of metabolites
-
Mamer OA, Tjoa SS, Scriver CR, Klassen GA 1976 Demonstration of a new mammalian isoleucine catabolic pathway yielding an R series of metabolites. Biochem J 160:417-426
-
(1976)
Biochem J
, vol.160
, pp. 417-426
-
-
Mamer, O.A.1
Tjoa, S.S.2
Scriver, C.R.3
Klassen, G.A.4
-
10
-
-
0017154417
-
Long-term management of a case of carbamyl phosphate synthetase deficiency using ketoanalogues and hydroxyanalogues of essential amino acids
-
Batshaw ML, Brusilow S, Walser M 1976 Long-term management of a case of carbamyl phosphate synthetase deficiency using ketoanalogues and hydroxyanalogues of essential amino acids. Pediatrics 58:227-235
-
(1976)
Pediatrics
, vol.58
, pp. 227-235
-
-
Batshaw, M.L.1
Brusilow, S.2
Walser, M.3
-
11
-
-
0000516952
-
Growth response of the rat to the keto analogues of leucine and isoleucine
-
Meister A, White J 1951 Growth response of the rat to the keto analogues of leucine and isoleucine. J Biol Chem 191:211-216
-
(1951)
J Biol Chem
, vol.191
, pp. 211-216
-
-
Meister, A.1
White, J.2
-
13
-
-
0026564150
-
On the mechanism of the formation of L-alloisoleucine and the 2-hydroxy-3-methylvaleric acid stereoisomers from L-isoleucine in maple syrup urine disease patients and in normal humans
-
Mamer OA, Reimer MLJ 1992 On the mechanism of the formation of L-alloisoleucine and the 2-hydroxy-3-methylvaleric acid stereoisomers from L-isoleucine in maple syrup urine disease patients and in normal humans. J Biol Chem 267:22141-22147
-
(1992)
J Biol Chem
, vol.267
, pp. 22141-22147
-
-
Mamer, O.A.1
Reimer, M.L.J.2
-
14
-
-
0018904353
-
Alloisoleucine formation in maple syrup urine disease: Isotopic evidence for the mechanism
-
Matthews DE, Ben-Galim E, Haymond MW, Bier DM 1980 Alloisoleucine formation in maple syrup urine disease: isotopic evidence for the mechanism. Pediatr Res 14:854-857
-
(1980)
Pediatr Res
, vol.14
, pp. 854-857
-
-
Matthews, D.E.1
Ben-Galim, E.2
Haymond, M.W.3
Bier, D.M.4
-
15
-
-
0028979695
-
Human branched-chain L-amino acid aminotransferase: Activity and subcellular localization in cultured skin fibroblasts
-
Schadewaldt P, Wendel U, Hammen H-W 1995 Human branched-chain L-amino acid aminotransferase: activity and subcellular localization in cultured skin fibroblasts. Amino Acids 9:147-160
-
(1995)
Amino Acids
, vol.9
, pp. 147-160
-
-
Schadewaldt, P.1
Wendel, U.2
Hammen, H.-W.3
-
16
-
-
0004837247
-
Studies on D-and L-α-keto-β-methylvaleric acids
-
Meister A 1951 Studies on D-and L-α-keto-β-methylvaleric acids. J Biol Chem 190:269-276
-
(1951)
J Biol Chem
, vol.190
, pp. 269-276
-
-
Meister, A.1
-
17
-
-
0017355846
-
Racemization and amination of the keto-analog of isoleucine in the intact dog
-
Weinberg RB, Walser M 1977 Racemization and amination of the keto-analog of isoleucine in the intact dog. Biochem Med 17:164-172
-
(1977)
Biochem Med
, vol.17
, pp. 164-172
-
-
Weinberg, R.B.1
Walser, M.2
-
19
-
-
0025307367
-
On the mechanism of L-alloisoleucine formation: Studies on a healthy subject and in fibroblasts from normals and patients with maple syrup urine disease
-
Schadewaldt P, Hammen H-W, Dalle-Feste C, Wendel U 1990 On the mechanism of L-alloisoleucine formation: studies on a healthy subject and in fibroblasts from normals and patients with maple syrup urine disease. J Inher Metab Dis 13:137-150
-
(1990)
J Inher Metab Dis
, vol.13
, pp. 137-150
-
-
Schadewaldt, P.1
Hammen, H.-W.2
Dalle-Feste, C.3
Wendel, U.4
-
20
-
-
0022460782
-
Resolution of D-and L-amino acids after precolumn derivatization with 0-phthalaldehyde by mixed chelation with Cu(II)-L-proline
-
Lam S 1986 Resolution of D-and L-amino acids after precolumn derivatization with 0-phthalaldehyde by mixed chelation with Cu(II)-L-proline. J Chromatogr 355:157-164
-
(1986)
J Chromatogr
, vol.355
, pp. 157-164
-
-
Lam, S.1
-
21
-
-
0026010306
-
Oral L-alloisoleucine loading studies in healthy subjects and in patients with maple syrup urine disease
-
Schadewaldt P, Dalle-Feste C, Langenbeck U, Wendel U 1991 Oral L-alloisoleucine loading studies in healthy subjects and in patients with maple syrup urine disease. Pediatr Res 30:430-434
-
(1991)
Pediatr Res
, vol.30
, pp. 430-434
-
-
Schadewaldt, P.1
Dalle-Feste, C.2
Langenbeck, U.3
Wendel, U.4
-
22
-
-
0000788257
-
Whole body branched-chain L-amino acid oxidation in overnight fasted human subjects
-
Bodner A, Hammen H-W, Renn W, Wendel U, Schadewaldt P 1997 Whole body branched-chain L-amino acid oxidation in overnight fasted human subjects. Isotopes Environ Health Stud 33:189-196
-
(1997)
Isotopes Environ Health Stud
, vol.33
, pp. 189-196
-
-
Bodner, A.1
Hammen, H.-W.2
Renn, W.3
Wendel, U.4
Schadewaldt, P.5
-
24
-
-
0024545076
-
Interrelation between the metabolism of L-isoleucine and L-allo-isoleucine in patients with maple syrup urine disease
-
Wendel U, Langenbeck U, Seakins JWT 1989 Interrelation between the metabolism of L-isoleucine and L-allo-isoleucine in patients with maple syrup urine disease. Pediatr Res 25:11-14
-
(1989)
Pediatr Res
, vol.25
, pp. 11-14
-
-
Wendel, U.1
Langenbeck, U.2
Seakins, J.W.T.3
-
25
-
-
0009554232
-
Studies in maple syrup urine disease
-
Dent CE, Westall RG 1961 Studies in maple syrup urine disease. Arch Dis Child 36:259-268
-
(1961)
Arch Dis Child
, vol.36
, pp. 259-268
-
-
Dent, C.E.1
Westall, R.G.2
-
26
-
-
0023635407
-
Utilization of the L-and DL-isomers of α-keto-β-methylvaleric acid by rats and comparative efficacy of the keto analogs of branched-chain amino acids provided as omithine, lysine and histidine salts
-
Funk MA, Lowry KR, Baker DH 1987 Utilization of the L-and DL-isomers of α-keto-β-methylvaleric acid by rats and comparative efficacy of the keto analogs of branched-chain amino acids provided as omithine, lysine and histidine salts. J Nutr 117:1550-1555
-
(1987)
J Nutr
, vol.117
, pp. 1550-1555
-
-
Funk, M.A.1
Lowry, K.R.2
Baker, D.H.3
-
28
-
-
0017661095
-
Isoenzyme patterns of branched-chain amino acid transaminase in human tissues and tumors
-
Goto M, Shinno H, Ichihara A 1977 Isoenzyme patterns of branched-chain amino acid transaminase in human tissues and tumors. Gann 68:663-667
-
(1977)
Gann
, vol.68
, pp. 663-667
-
-
Goto, M.1
Shinno, H.2
Ichihara, A.3
-
29
-
-
0031750339
-
A molecular model of human branched-chain amino acid metabolism
-
Suryawan A, Hawes JW, Harris RA, Shimomura Y, Jenkins AE, Hutson SM 1998 A molecular model of human branched-chain amino acid metabolism. Am J Clin Nutr 68:72-81
-
(1998)
Am J Clin Nutr
, vol.68
, pp. 72-81
-
-
Suryawan, A.1
Hawes, J.W.2
Harris, R.A.3
Shimomura, Y.4
Jenkins, A.E.5
Hutson, S.M.6
-
30
-
-
0024215484
-
Measurement of plasma and tissue levels of branched-chain a-keto acids by gas-liquid chromatography
-
Crowell PL, Miller RH, Harper AE 1988 Measurement of plasma and tissue levels of branched-chain a-keto acids by gas-liquid chromatography. Methods Enzymol 166:39-46
-
(1988)
Methods Enzymol
, vol.166
, pp. 39-46
-
-
Crowell, P.L.1
Miller, R.H.2
Harper, A.E.3
-
31
-
-
0027314007
-
Arteriovenous differences and tissue concentrations of branched-chain ketoacids
-
Matuso Y, Yagi M, Walser M 1993 Arteriovenous differences and tissue concentrations of branched-chain ketoacids J Lab Clin Med 121:779-784
-
(1993)
J Lab Clin Med
, vol.121
, pp. 779-784
-
-
Matuso, Y.1
Yagi, M.2
Walser, M.3
-
32
-
-
0018860321
-
Muscle and plasma amino acids following injury: Influence of intercurrent infections
-
Askanazi J, Carpentier YA, Michelsen CB, Elwyn DH, Furst P, Kantrowitz LR, Gump FE, Kinney JM 1980 Muscle and plasma amino acids following injury: influence of intercurrent infections. Ann Surg 192:78-85
-
(1980)
Ann Surg
, vol.192
, pp. 78-85
-
-
Askanazi, J.1
Carpentier, Y.A.2
Michelsen, C.B.3
Elwyn, D.H.4
Furst, P.5
Kantrowitz, L.R.6
Gump, F.E.7
Kinney, J.M.8
-
33
-
-
0030023284
-
Infusion of labeled K1C is more accurate than labeled leucine to determine human muscle protein synthesis
-
Chinkes D, Klein S, Zhang X-J, Wolfe RR 1996 Infusion of labeled K1C is more accurate than labeled leucine to determine human muscle protein synthesis. Am J Physiol 270:E67-E71
-
(1996)
Am J Physiol
, vol.270
-
-
Chinkes, D.1
Klein, S.2
Zhang, X.-J.3
Wolfe, R.R.4
-
34
-
-
0023863055
-
Transamination and oxidative decarboxylation rates of branched-chain 2-oxo acids in cultured human skin fibroblasts
-
Schadewaldt P, Radeck W, Hammen H-W, Wendel U 1988 Transamination and oxidative decarboxylation rates of branched-chain 2-oxo acids in cultured human skin fibroblasts. Pediatr Res 23:40-44
-
(1988)
Pediatr Res
, vol.23
, pp. 40-44
-
-
Schadewaldt, P.1
Radeck, W.2
Hammen, H.-W.3
Wendel, U.4
-
35
-
-
0024340092
-
Transamination and oxidative decarboxylation of L-isoleucine, L-alloisoleucine and related 2-oxo acids in perfused rat hind limb muscle
-
Schadewaldt P, Radeck W, Hammen H-W, Staib W 1989 Transamination and oxidative decarboxylation of L-isoleucine, L-alloisoleucine and related 2-oxo acids in perfused rat hind limb muscle. Biochim Biophys Acta 992:115-123
-
(1989)
Biochim Biophys Acta
, vol.992
, pp. 115-123
-
-
Schadewaldt, P.1
Radeck, W.2
Hammen, H.-W.3
Staib, W.4
-
36
-
-
0343027673
-
Über das vorkommen von äthylmalonsäure im harn
-
Stalder K 1959 Über das Vorkommen von Äthylmalonsäure im Harn. Hoppe-Seyler's Z Physiol Chem 314:205-210
-
(1959)
Hoppe-Seyler's Z Physiol Chem
, vol.314
, pp. 205-210
-
-
Stalder, K.1
-
37
-
-
0032771536
-
Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease
-
Schadewaldt P, Hammen H-W, Ott A-C, Wendel U 1999 Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease. J Inherit Metab Dis 22:706-722
-
(1999)
J Inherit Metab Dis
, vol.22
, pp. 706-722
-
-
Schadewaldt, P.1
Hammen, H.-W.2
Ott, A.-C.3
Wendel, U.4
|