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72715

Sigma-Aldrich

Glutaryl-L-carnitine lithium salt

≥98.0% (TLC)

Synonym(s):

L-Carnitine lithium glutarate

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About This Item

Empirical Formula (Hill Notation):
C12H21NO6 · xLi+
CAS Number:
102636-82-8
Molecular Weight:
275.30 (free acid basis)
UNSPSC Code:
41116107
PubChem Substance ID:
329764299
NACRES:
NA.25

product name

Glutaryl-L-carnitine lithium salt, ≥98.0% (TLC)

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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Product No.
Description
Pricing

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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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
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
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
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