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Merck

06689

3-Methylglutaconic acid, mixture of E and Z isomers

≥98.0% (HPLC)

Sinonimo/i:

3-Methyl-2-pentenedioic acid, NSC 249232

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Informazioni su questo articolo

Formula empirica (notazione di Hill):
C6H8O4
Numero CAS:
5746-90-7
Peso molecolare:
144.13
UNSPSC Code:
41116107
NACRES:
NA.24
PubChem Substance ID:
329748259
MDL number:
MFCD01556044
Beilstein/REAXYS Number:
1722907

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Nome del prodotto

3-Methylglutaconic acid, mixture of E and Z isomers, ≥98.0% (HPLC)

InChI key

WKRBKYFIJPGYQC-DUXPYHPUSA-N

SMILES string

C/C(CC(O)=O)=C\C(O)=O

InChI

1S/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+

assay

≥98.0% (HPLC)

application(s)

clinical testing

format

neat

storage temp.

2-8°C

Quality Level

100

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Questo articolo
441088.002958.14659
3-Methylglutaconic acid, mixture of E and Z isomers ≥98.0% (HPLC)

Supelco

06689

3-Methylglutaconic acid, mixture of E and Z isomers

(E)-3-Methylglutaconic acid ≥97.0% (HPLC)

Supelco

44108

(E)-3-Methylglutaconic acid

Glutaric acid for synthesis

Sigma-Aldrich

8.00295

Glutaric acid

Dimethyl glutarate for synthesis

Sigma-Aldrich

8.14659

Dimethyl glutarate

format

neat

format

neat

format

-

format

-

assay

≥98.0% (HPLC)

assay

≥97.0% (HPLC)

assay

≥99.0% (acidimetric)

assay

-

application(s)

clinical testing

application(s)

clinical testing

application(s)

-

application(s)

-

storage temp.

2-8°C

storage temp.

2-8°C

storage temp.

2-30°C

storage temp.

2-30°C

Quality Level

100

Quality Level

100

Quality Level

200

Quality Level

200

Biochem/physiol Actions

3-Methylglutaconic acid is a metabolite (as the CoA thioester) in the leucine degradative pathway as well as the mevalonate shunt, a pathway that links isoprenoid metabolism with mitochondrial acetyl-CoA metabolism. 3-Methylglutaconic acid accumulates in patients with a deficiency of 3-methylglutaconyl-CoA hydratase.

Classe di stoccaggio

11 - Combustible Solids

wgk

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

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Certificati d'analisi (COA)

Lot/Batch Number
BCBN6472

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E Holme et al.
Pediatric research, 32(6), 731-735 (1992-12-01)
We report the finding of mitochondrial ATP-synthase deficiency in a child with persistent 3-methylglutaconic aciduria. The child presented in the neonatal period with severe lactic acidosis, which was controlled by Na-HCO3 and glucose infusions. During the 1st y of life
R I Kelley
Clinica chimica acta; international journal of clinical chemistry, 220(2), 157-164 (1993-11-15)
A method is described for quantification of the trace metabolite, 3-methylglutaconic acid, by isotope-dilution gas chromatography/mass spectrometry using synthetic 3-[2,4,6-13C3]methylglutaconic acid. Results are shown for quantification of 3-methylglutaconic acid in plasma, urine, cerebrospinal fluid and amniotic fluid for both normal
R I Kelley et al.
Pediatric research, 37(5), 671-674 (1995-05-01)
The branched-chain organic acid, 3-methylglutaconic acid, is an intermediate (as the CoA thioester) in the leucine degradative pathway as well as the mevalonate shunt, a pathway that links isoprenoid metabolism with mitochondrial acetyl-CoA metabolism. Because the majority of patients with
G A Mitchell et al.
Prenatal diagnosis, 15(8), 725-729 (1995-08-01)
We report the first molecular prenatal diagnosis of 3-hydroxy-3-methylglutaryl CoA lyase (HL) deficiency. The proband had a classic but severe presentation with hypoketotic hypoglycaemia and acidosis, secondary mental retardation, and epilepsy, and HL deficiency was documented in cultured fibroblasts. We
H Costeff et al.
Annals of neurology, 33(1), 103-104 (1993-01-01)
Behr's syndrome consists of recessively inherited infantile optic atrophy, together with chronic neurological disturbances such as ataxia, extrapyramidal dysfunction, and juvenile spastic paresis. This syndrome was found to be relatively common among Iraqi Jews. For our study, 18 such patients
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