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Merck
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주요 문서

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72715

Sigma-Aldrich

Glutaryl-L-carnitine lithium salt

≥98.0% (TLC)

동의어(들):

L-Carnitine lithium glutarate

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About This Item

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

제품명

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

Quality Level

100

분석

≥98.0% (TLC)

양식

solid

광학 활성

[α]/D -20±2°, c = 1 in H2O

불순물

≤10% water

색상

white to off-white

저장 온도

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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관련 카테고리

생화학적/생리학적 작용

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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제품 번호
설명
가격

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable

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시험 성적서(COA)

Lot/Batch Number
BCCB1763
BCBQ0141V
BCBQ0141

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문서 라이브러리 방문

Glutaric aciduria; a "new" disorder of amino acid metabolism.
S I Goodman et al.
Biochemical medicine, 12(1), 12-21 (1975-01-01)
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
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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