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72715

Sigma-Aldrich

Glutaryl-L-carnitine lithium salt

≥98.0% (TLC)

Sinonimo/i:

L-Carnitine lithium glutarate

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About This Item

Formula empirica (notazione di Hill):
C12H21NO6 · xLi+
Numero CAS:
102636-82-8
Peso molecolare:
275.30 (free acid basis)
Codice UNSPSC:
41116107
ID PubChem:
329764299
NACRES:
NA.25

product name

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

Livello qualitativo

100

Saggio

≥98.0% (TLC)

Forma fisica

solid

Attività ottica

[α]/D -20±2°, c = 1 in H2O

Impurezze

≤10% water

Colore

white to off-white

Temperatura di conservazione

2-8°C

Stringa SMILE

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
NXJAXUYOQLTISD-SECBINFHSA-N

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Categorie correlate

Azioni biochim/fisiol

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.

Comunemente ordinati con questo prodotto

N° Catalogo
Descrizione
Determinazione del prezzo

Codice della classe di stoccaggio

11 - Combustible Solids

Classe di pericolosità dell'acqua (WGK)

WGK 3

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable

Certificati d'analisi (COA)

Cerca il Certificati d'analisi (COA) digitando il numero di lotto/batch corrispondente. I numeri di lotto o di batch sono stampati sull'etichetta dei prodotti dopo la parola ‘Lotto’ o ‘Batch’.

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[(3R)-3-Hydroxyhexanoyl]-L-carnitine analytical standard

Supelco

54253

[(3R)-3-Hydroxyhexanoyl]-L-carnitine

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
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
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
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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