All Photos(1)
Synonym(s):
N-(3-Methyl-1-oxobutyl)glycine, N-(3-Methylbutanoyl)glycine, [(3-Methylbutanoyl)amino]acetic acid
Empirical Formula (Hill Notation):
C7H13NO3
CAS Number:
Molecular Weight:
159.18
Beilstein:
1766622
MDL number:
UNSPSC Code:
12352209
PubChem Substance ID:
NACRES:
NA.26
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Quality Level
Assay
≥97.0% (GC)
form
powder or crystals
color
white to off-white
mp
87-90 °C
format
neat
InChI
1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11)
InChI key
ZRQXMKMBBMNNQC-UHFFFAOYSA-N
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Biochem/physiol Actions
Isovalerylglycine is a byproduct of the catabolism of the amino acid leucine. Accumulation of isovalerylglycine occurs in Isovaleric Acidemia (IVA), an autosomal recessive disorder caused by mutations in the isovaleryl-CoA dehydrogenase gene. The deficiency of this enzyme in the metabolism of leucine leads to the accumulation of a series of isovaleryl-CoA metabolites, such as isovalerylglycine (IVG).
WGK
WGK 3
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
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D G Hine et al.
Pediatric research, 20(3), 222-226 (1986-03-01)
A stable isotope dilution method was developed to measure accurately small amounts of isovalerylglycine in amniotic fluid and urine for the prenatal diagnosis of isovaleric acidemia. [4,4,4-D3]Isovalerylglycine was synthesized and used as an internal standard. Samples were extracted, methylated, and
Yosuke Shigematsu et al.
Clinica chimica acta; international journal of clinical chemistry, 386(1-2), 82-86 (2007-09-14)
Recent neonatal screening for isovaleric acidemia by tandem mass spectrometry based on dried blood-spot levels of C5-acylcarnitines, including isovalerylcarnitine and its isomer, pivaloylcarnitine, which is derived from pivalate-generating antibiotics, has caused many false-positive results. We have developed a method to
S I Goodman et al.
Pediatric research, 14(1), 12-17 (1980-01-01)
When amino acids were infused at a rate of 4 g/kg/day, an infant with hypoglycemia, metabolic acidemia and chronic regurgitation showed hypersarcosinemia and excreted abnormal amounts of sarcosine, isovalerylglycine, isobutyrylglycine, alpha-methylbutyrylglycine, and beta-hydroxyisovaleric, glutaric, alpha-hydroxyglutaric, methylsuccinic, and alpha-hydroxyisobutyric acids in
M H Fries et al.
The Journal of pediatrics, 129(3), 449-452 (1996-09-01)
To assess the effectiveness of glycine and carnitine therapy on isovaleryl conjugate excretion in isovaleric acidemia (IVA). Urinary isovalerylglycine (IVG) and isovalerylcarnitine (IVC) were measured from 12-hour urine specimens collected overnight from an 8-year-old patient with IVA (who had no
C D Capo-chichi et al.
The American journal of clinical nutrition, 69(4), 672-678 (1999-04-10)
Thyroid hormones, riboflavin, riboflavin cofactors, and organic acids were assessed in girls with anorexia nervosa. The objective was to examine the effect of malnutrition and low thyroid hormone concentrations on erythrocyte and plasma riboflavin metabolism and their relation with urinary
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