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About This Item
Empirical Formula (Hill Notation):
C13H23NO6
CAS Number:
Molecular Weight:
289.32
MDL number:
UNSPSC Code:
12352211
NACRES:
NA.25
Product Name
3-Me-Glutaryl Carnitine, Avanti Research™ - A Croda Brand 870855A, paste
SMILES string
C[N+](C)(C)C[C@H](OC(CC(C)CC(O)=O)=O)CC([O-])=O
InChI
1S/C13H23NO6/c1-9(5-11(15)16)6-13(19)20-10(7-12(17)18)8-14(2,3)4/h9-10H,5-8H2,1-4H3,(H-,15,16,17,18)
InChI key
HFCPFJNSBPQJDP-UHFFFAOYSA-N
assay
>99% (TLC)
form
paste
packaging
pkg of 1 × 5 mg (870855A-5mg)
manufacturer/tradename
Avanti Research™ - A Croda Brand 870855A
lipid type
bioactive lipids
shipped in
dry ice
storage temp.
−20°C
Biochem/physiol Actions
3-Me-Glutaryl Carnitine metabolite may be found in urine samples. The detection of 3-methylglutarylcarnitine and other metabolites may play a role in a treatment strategy for carnitine deficiency.
Packaging
5 mL Clear Glass Sealed Ampule (870855A-5mg)
Legal Information
Avanti Research is a trademark of Avanti Polar Lipids, LLC
Storage Class
10 - Combustible liquids
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S Jooste et al.
Clinica chimica acta; international journal of clinical chemistry, 230(1), 1-8 (1994-10-14)
Two patients, diagnosed with 3-methylglutaconic aciduria, who presented with diverse clinical and metabolic manifestations, were studied. Glycine conjugation as a possible detoxification mechanism in these two patients yielded negative results. Carnitine conjugates were however detected. 3-Methylglutarylcarnitine was observed in the
C R Roe et al.
The Journal of clinical investigation, 77(4), 1391-1394 (1986-04-01)
Deficiency of 3-hydroxy-3-methylglutaryl-coenzyme A (CoA) lyase affects the metabolism of leucine as well as ketogenesis. This disorder is one of an increasing list of inborn errors of metabolism that presents clinically like Reye's Syndrome or nonketotic hypoglycemia. Four patients with
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