M47604
3-甲基戊烯二酸
99%
别名:
β-Methylglutaric acid, 3-Methylpentanedioic acid
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关于此项目
线性分子式:
CH3CH(CH2COOH)2
化学文摘社编号:
分子量:
146.14
UNSPSC Code:
12352100
NACRES:
NA.22
PubChem Substance ID:
EC Number:
210-951-5
Beilstein/REAXYS Number:
1759502
MDL number:
产品名称
3-甲基戊烯二酸, 99%
InChI key
XJMMNTGIMDZPMU-UHFFFAOYSA-N
InChI
1S/C6H10O4/c1-4(2-5(7)8)3-6(9)10/h4H,2-3H2,1H3,(H,7,8)(H,9,10)
SMILES string
CC(CC(O)=O)CC(O)=O
assay
99%
mp
81-86 °C (lit.)
Quality Level
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存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
dust mask type N95 (US), Eyeshields, Gloves
D Chitayat et al.
Journal of inherited metabolic disease, 15(2), 204-212 (1992-01-01)
The Mendelian disorder known as 3-methylgutaconic aciduria (McKusick 250950) gives evidence of allelic and locus heterogeneity. Type 1 has a mild clinical phenotype and confirmed 3-methylgutaconyl-CoA hydratase deficiency; inheritance is autosomal recessive. Other forms have major clinical manifestations and subdivide
E A Haan et al.
European journal of pediatrics, 146(5), 484-488 (1987-09-01)
Persistent excretion of 3-methylglutaconic acid was found in a 6-month-old infant with multiple minor physical malformations and delayed development. During two episodes of intercurrent viral illness, the patient developed severe metabolic acidosis and excreted large amounts of lactate, 3-hydroxybutyrate and
R I Kelley et al.
The Journal of pediatrics, 119(5), 738-747 (1991-11-01)
Seven boys with an apparently X-linked syndrome of dilated cardiomyopathy, growth retardation, neutropenia, and persistently elevated urinary levels of 3-methylglutaconate, 3-methylglutarate, and 2-ethylhydracrylate were studied. The natural history of the disorder was characterized by severe or lethal cardiac disease and
M Duran et al.
The Journal of pediatrics, 101(4), 551-554 (1982-10-01)
Two brothers, aged 7 and 5 years, who excreted large amounts of the leucine metabolites 3-methylglutaconic acid, 3-methylglutaric acid, and 3-hydroxyisovaleric acid, are described. The excretion of these metabolites could be enhanced by increasing the leucine intake. Restriction of the
B Hagberg et al.
Clinica chimica acta; international journal of clinical chemistry, 134(1-2), 59-67 (1983-10-31)
We studied two children who developed normally for the first 3-4 months of life and then displayed a failure-to-thrive syndrome, regression in psychomotor development, pronounced muscular hypotonia, and liver damage. At the age of about 1-2 years, optic atrophy and
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