Fortfahren mit
44108
(E)-3-Methyl-glutaconsäure
≥97.0% (HPLC)
Synonym(e):
(2E)-3-Methyl-2-pentendisäure
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| Packungsgröße | SKU | Verfügbarkeit | Preis |
|---|---|---|---|
| 50 mg | Warenkorb auf Verfügbarkeit prüfen | € 174,00 |
Über diesen Artikel
Empirische Formel (Hill-System):
C6H8O4
CAS-Nummer:
Molekulargewicht:
144.13
UNSPSC Code:
41116107
PubChem Substance ID:
NACRES:
NA.24
Beilstein/REAXYS Number:
1722909
MDL number:
Technischer Dienst
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Unterstützung erhaltenQuality Level
assay
≥97.0% (HPLC)
mp
137-143 °C
application(s)
clinical testing
format
neat
storage temp.
2-8°C
SMILES string
OC(=O)C\C(=C\C(=O)O)\C
InChI
1S/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+
InChI key
WKRBKYFIJPGYQC-DUXPYHPUSA-N
Biochem/physiol Actions
3-Methylglutaconic aciduria (MGCA) is a group of metabolic disorders characterized by increased urinary excretion of 3-methylglutaconic acid and 3-methylglutaric acid. Five distinct forms of MGCA have been recognized. MGCA type 1 is caused by primary deficiency of the mitochondrial enzyme 3-methylglutaconyl-CoA hydratase (3-MGCH), resulting in a block of leucine degradation. In all other types, the activities of 3-MGCH and other enzymes of leucine degradation are normal and the MGCA is thought to be secondary to a defect in another pathway. MGCA type 2, also known as Barth syndrome, is an X-linked cardiomyopathy associated with skeletal myopathy, neutropenia, and growth retardation. MGCA type 3, also referred to as Costeff optic atrophy syndrome, is an autosomal recessive disorder caused by mutations in the gene OPA3. It is characterized by early-onset bilateral optic atrophy, later-onset extrapyramidal dysfunction. MGCA type 5 caused by mutation in the DNAJC19 gene, is characterized by early-onset dilated cardiomyopathy with conduction defects, nonprogressive cerebellar ataxia, testicular dysgenesis, and growth failure. MGCA type 4 is the unclassified type, which includes all other patients with MGCA.
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Dieser Artikel | |
|---|---|
| format neat | format neat |
| assay ≥97.0% (HPLC) | assay ≥95.0% (GC), 90.0-105.0% (T) |
| storage temp. 2-8°C | storage temp. 2-8°C |
| application(s) clinical testing | application(s) clinical testing |
| Quality Level 100 | Quality Level 100 |
| mp 137-143 °C | mp - |
signalword
Warning
hcodes
Hazard Classifications
Eye Irrit. 2 - Skin Irrit. 2
Lagerklasse
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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Rebecca Young et al.
Biochemical and biophysical research communications, 534, 261-265 (2020-12-08)
3-methylglutaconic (3MGC) aciduria is associated with a growing number of discrete inborn errors of metabolism. Herein, an antibody-based approach to detection/quantitation of 3MGC acid has been pursued. When trans-3MGC acid conjugated keyhole limpet hemocyanin (KLH) was inoculated into rabbits a
Solution of a classical problem. Tautomerism and isomerism in the α-methylglutaconic acid series
Kagan, J., et al.
The Journal of Organic Chemistry, 40, 3085-3093 (1975)
M Duran et al.
Biomedical mass spectrometry, 9(1), 1-5 (1982-01-01)
The identification of (E)-2-methylglutaconic acid, a 'new' metabolite of isoleucine, is described. The substance was detected in urine samples from patients with propionic acidaemia, methylmalonic acidaemia and so-called beta-ketothiolase deficiency; in the majority of cases together with N-tiglylglycine. (E)-2-Methylglutaconic acid
Global Trade Item Number
| SKU | GTIN |
|---|---|
| 44108-50MG | 04061832281834 |
| 44108-10MG | 04061833379639 |
| 44108-250MG | 04061833471777 |