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Maple Syrup Urine Disease
Known as:
Branched-Chain Ketoacidurias
, BRANCHED-CHAIN KETONURIA
, Branched Chain Ketoaciduria
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An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of…
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National Institutes of Health
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Related topics
Related topics
42 relations
3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
Amino Acids, Branched-Chain
Amino acidemia disorder suspected:Prid:Pt:Bld.dot:Nom
Ataxia
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Broader (3)
Amino Acid Metabolism, Inborn Errors
Ketonuria
Mental Retardation
Narrower (4)
Intermediate Maple Syrup Urine Disease
Intermittent Maple Syrup Urine Disease
Lactic Acidosis, Congenital Infantile, Due To Lad Deficiency
MAPLE SYRUP URINE DISEASE, TYPE II
Papers overview
Semantic Scholar uses AI to extract papers important to this topic.
2005
2005
Biosynthesis of blood-group I and i substances. Specificity of bovine colostrum beta-N-acetyl-D-glucosaminide beta 1 leads to 4 galactosyltransferase.
W. M. Blanken
,
G. Hooghwinkel
,
D. H. van den Eijnden
European Journal of Biochemistry
2005
Corpus ID: 22538566
Highly purified bovine colostrum beta-N-acetylglucosaminide beta 1 leads to 4 galactosyltransferase was used to investigate the…
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2000
2000
Assessing the Non-Timber Value of Forests: A Revealed-Preference, Hedonic Model
R. Scarpa
,
J. Buongiorno
,
Jiing-Shyang Hseu
,
K. Abt
2000
Corpus ID: 152838609
Based on revealed preference theory, the value of non-timber goods and services obtained by forest owners, private or public…
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1995
1995
Maple Syrup Urine Disease
M. Knaap
,
J. Valk
1995
Corpus ID: 73864282
Maple syrup urine (MSUD) is a heterogeneous disorder. Classification is based on clinical presentation and outcome. Clinically…
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Highly Cited
1988
Highly Cited
1988
Temporal and Spatial Regulation of Plant Genes
D. Verma
,
R. Goldberg
Plant Gene Research
1988
Corpus ID: 39910649
First attempts to isolate plant genes were for those genes that are abun dantly expressed in a particular plant organ at a…
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1988
1988
Influence of amino acid administration on whole-body leucine kinetics and resting metabolic rate in postabsorptive normal subjects.
P. Pacy
,
J. Garrow
,
G. C. Ford
,
H. Merritt
,
David Halliday
Clinical science
1988
Corpus ID: 35889749
1. We have investigated the effect of an amino acid mixture (Vamin 14; 57.4 +/- 10.2 mumol h-1 kg-1) on whole-body leucine…
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1987
1987
Maple syrup urine disease in five hereford calves in ontario.
Baird Jd
,
Wojcinski Zw
,
Wise Ap
,
Godkin Ma
1987
Corpus ID: 58717371
Five newborn grade horned or polled Hereford calves in Ontario exhibited the clinical signs characteristic of hereditary…
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1983
1983
Synthesis and myocardial kinetics of N-13 and C-11 labeled branched-chain L-amino acids.
J. Barrio
,
F. J. Baumgartner
,
+6 authors
F. Liu
Journal of Nuclear Medicine
1983
Corpus ID: 19392748
Glutamate dehydrogenase (GDH), immobilized on CNBr-activated Sepharose supports, was used with N-13 ammonia to aminate alpha…
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Highly Cited
1980
Highly Cited
1980
Purification and properties of a binding protein for branched-chain amino acids in Pseudomonas aeruginosa
T. Hoshino
,
M. Kageyama
Journal of Bacteriology
1980
Corpus ID: 20561627
A binding protein for branched-chain amino acids was purified to a homogeneous state from shock fluid of Pseudomonas aeruginosa…
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1979
1979
Valine-toxic intermittent maple syrup urine disease: a previously unrecognized variant.
W. Zipf
,
V. Hieber
,
R. Allen
Pediatrics
1979
Corpus ID: 34521821
An 18-month-old boy with repeated episodes of vomiting, lethargy, irritability, acidosis, and hypoglycemia from the age of 2…
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1963
1963
Heterozygoten-Test fr die Ahornsirupkrankheit: Maple Syrup Urine Disease
F. Linneweh
,
M. Ehrlich
1963
Corpus ID: 85710116
Bei drei heterozygoten Elternpaaren von drei Kranken mit Ahornsirupkrankheit wurde saulenchromatographisch teils schon im…
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