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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:
102636-82-8
Molecular Weight:
275.30 (free acid basis)
UNSPSC Code:
12352200
PubChem Substance ID:
329764299
NACRES:
NA.25

Quality Level

100

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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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.

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WGK

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Flash Point(C)

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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
Stefan Kölker et al.
Annals of neurology, 55(1), 7-12 (2004-01-06)
Glutaryl-CoA dehydrogenase deficiency is an inherited organic aciduria with predominantly neurological presentation. Biochemically, it is characterized by an accumulation and enhanced urinary excretion of two key organic acids, glutaric acid and 3-hydroxyglutaric acid. If untreated, acute striatal degeneration is often
Glutaric aciduria; a "new" disorder of amino acid metabolism.
S I Goodman et al.
Biochemical medicine, 12(1), 12-21 (1975-01-01)
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
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
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