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2-methylbutyrylglycine
Known as:
Glycine, N-(2-methyl-1-oxobutyl)-
National Institutes of Health
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2 relations
Broader (1)
Glycine
analogs & derivatives
Papers overview
Semantic Scholar uses AI to extract papers important to this topic.
2019
2019
Improved clinical outcome following liver transplant in patients with ethylmalonic encephalopathy
Allison Tam
,
Noura S AlDhaheri
,
+8 authors
F. Scaglia
American journal of medical genetics. Part A
2019
Corpus ID: 76662607
Ethylmalonic encephalopathy (EE) is a rapidly progressive autosomal recessive mitochondrial disease caused by biallelic…
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2007
2007
Ethylmalonic encephalopathy: clinical and biochemical observations.
D. Zafeiriou
,
P. Augoustides-Savvopoulou
,
+9 authors
N. Gregersen
Neuropediatrics
2007
Corpus ID: 42167906
Ethylmalonic encephalopathy (EE) is a rare, recently defined inborn error of metabolism which affects the brain, gastrointestinal…
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2005
2005
2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation.
S. Korman
,
B. Andresen
,
A. Zeharia
,
A. Gutman
,
A. Boneh
,
J. Pitt
Clinical chemistry
2005
Corpus ID: 11717401
BACKGROUND Isolated excretion of 2-methylbutyrylglycine (2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase…
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2005
2005
The occurrence of C6–C10-dicarboxylic acids, ethylmalonic acid, 5-hydroxycaproic acid, butyrylglycine, isovalerylglycine, isobutyrylglycine, 2-methylbutyrylglycine and glutaric acid in the urine of…
N. Gregersen
,
S. Kolvraa
Journal of Inherited Metabolic Disease
2005
Corpus ID: 46198130
C6-Clo-Dicarboxylic aciduria may be caused by an inborn error of fatty acid 13-oxidation. In the cases reported the defect has…
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Highly Cited
2000
Highly Cited
2000
2-Methylbutyryl-Coenzyme A Dehydrogenase Deficiency: A New Inborn Error of L-Isoleucine Metabolism
K. Gibson
,
T. Burlingame
,
+13 authors
J. Vockley
Pediatric Research
2000
Corpus ID: 898859
An 4-mo-old male was found to have an isolated increase in 2-methylbutyrylglycine (2-MBG) and 2-methylbutyrylcarnitine (2-MBC) in…
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Highly Cited
1999
Highly Cited
1999
Short-chain acyl–CoA dehydrogenase deficiency
I. Tein
,
R. Haslam
,
W. Rhead
,
M. Bennett
,
L. E. Becker
,
J. Vockley
Neurology
1999
Corpus ID: 23381938
Objective: To determine an underlying genetic defect within the differential diagnosis of congenital multicore myopathy…
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1998
1998
Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism.
M. Nowaczyk
,
D. Lehotay
,
+4 authors
J. Clarke
Metabolism: clinical and experimental
1998
Corpus ID: 40778417
1980
1980
Excretion of short-chain N-acylglycines in the urine of a patient with D-glyceric acidemia.
S. Kolvraa
,
N. Gregersen
,
N. Brandt
Clinica chimica acta; international journal of…
1980
Corpus ID: 1533793
Highly Cited
1978
Highly Cited
1978
Abnormal metabolites of isoleucine in a patient with propionyl-CoA carboxylase deficiency.
L. Sweetman
,
W. Weyler
,
W. Nyhan
,
C. D. de Céspedes
,
A. R. Loria
,
Y. Estrada
Biomedical mass spectrometry
1978
Corpus ID: 11360367
A number of previously unrecognized abnormal metabolites have been identified and quantitated in the urine of a patient with an…
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1972
1972
The identification of tiglylglycine in the urine of a child with -methylcrotonylglycinuria.
D. Gompertz
,
G. Draffan
Clinica chimica acta; international journal of…
1972
Corpus ID: 27997000
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