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72715

Sigma-Aldrich

Glutaryl-L-carnitine lithium salt

≥98.0% (TLC)

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Synonym(s):
L-Carnitine lithium glutarate
Empirical Formula (Hill Notation):
C12H21NO6 · xLi+
CAS Number:
Molecular Weight:
275.30 (free acid basis)
PubChem Substance ID:
NACRES:
NA.25

Quality Level

Assay

≥98.0% (TLC)

form

solid

optical activity

[α]/D -20±2°, c = 1 in H2O

impurities

≤10% water

color

white to off-white

storage temp.

2-8°C

SMILES string

C[N+](C)(C)C[C@H](OC(CCCC(O)=O)=O)CC([O-])=O.C

InChI

1S/C12H21NO6/c1-13(2,3)8-9(7-11(16)17)19-12(18)6-4-5-10(14)15/h9H,4-8H2,1-3H3,(H-,14,15,16,17)/t9-/m1/s1

InChI key

NXJAXUYOQLTISD-SECBINFHSA-N

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1 of 4

This Item
91499646091745472
form

solid

form

-

form

solid

form

solid

optical activity

[α]/D -20±2°, c = 1 in H2O

optical activity

[α]/D -25.0 to -19.0°, c = 0.1 in H2O

optical activity

-

optical activity

-

impurities

≤10% water

impurities

-

impurities

-

impurities

-

color

white to off-white

color

-

color

-

color

-

storage temp.

2-8°C

storage temp.

2-8°C

storage temp.

2-8°C

storage temp.

2-8°C

Biochem/physiol Actions

Increased formation and excretion of glutarylcarnitin results from a glutaryl-CoA dehydrogenase deficiency, an inborn error of lysine and tryptophan metabolism. Secondary carnitine depletion due to increased formation and urinary excretion of glutarylcarnitine is suggested to play an important role in the neuropathogenesis of glutaryl-CoA dehydrogenase deficiency, inducing excitotoxic neuronal damage and mitochondrial dysfunction.

also commonly purchased with this product

Product No.
Description
Pricing

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable


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Butyryl-L-carnitine ≥97.0% (TLC)

Sigma-Aldrich

42623

Butyryl-L-carnitine

Hexanoyl-L-carnitine ≥97.0% (TLC)

Sigma-Aldrich

07439

Hexanoyl-L-carnitine

Hee Su Kim et al.
Annals of clinical and laboratory science, 44(2), 213-216 (2014-05-06)
Mutations in the glutaryl-CoA dehydrogenase gene can result in Glutaric aciduria type 1(GA 1) by accumulation of glutaric acid, 3-hydroxyglutaric acid (3-OH-GA), and glutarylcarnitine (C5DC). GA 1 is characterized by macrocephaly, subdural hemorrhage (SDH), and dystonic movement disorder after acute
Glutaric aciduria; a "new" disorder of amino acid metabolism.
S I Goodman et al.
Biochemical medicine, 12(1), 12-21 (1975-01-01)
Kim M Huffman et al.
Diabetes care, 32(9), 1678-1683 (2009-06-09)
To determine whether circulating metabolic intermediates are related to insulin resistance and beta-cell dysfunction in individuals at risk for type 2 diabetes. In 73 sedentary, overweight to obese, dyslipidemic individuals, insulin action was derived from a frequently sampled intravenous glucose
Chee-Seng Lee et al.
Metabolic brain disease, 28(1), 61-67 (2012-10-30)
Glutaric aciduria type I (GA-I) is an inborn error of lysine and tryptophan metabolism. Clinical manifestations of GA-I include dystonic or dyskinetic cerebral palsy, but when the symptoms occur, treatment is not effective. In Taiwan, newborn screening for GA-I started
K Kidouchi et al.
Clinica chimica acta; international journal of clinical chemistry, 164(3), 261-266 (1987-05-15)
A technique for the identification of glutarylcarnitine in urine from a patient with glutaric aciduria type 1 is described. The patient's urine sample was partially purified using an anion exchange column and analyzed by a carboxylic acid analyzer fitted with

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