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L-Alloisoleucine ((3R)-LS-Isoleucine) Sale

(Synonyms: L-别异亮氨酸; (3R)-LS-Isoleucine; L-Allo-isoleucine) 目录号 : GC30624

A branched-chain amino acid

L-Alloisoleucine ((3R)-LS-Isoleucine) Chemical Structure

Cas No.:1509-34-8

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25mg
¥446.00
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Sample solution is provided at 25 µL, 10mM.

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L-Alloisoleucine is a branched-chain amino acid and diastereomer of L-isoleucine.1 It is formed from L-isoleucine by transamination.2 Plasma L-alloisoleucine levels are increased in patients with maple syrup urine disease (MSUD), an inborn error of metabolism characterized by a deficiency in the branched-chain α-ketoacid dehydrogenase complex, the complex that catalyzes the degradation of branched-chain amino acids, leading to the accumulation of branched-chain amino acids in the plasma and urine, a maple syrup-like odor in the urine, and neurological impairments.1

1.Schadewaldt, P., Bodner-Leidecker, A., Hammen, H.W., et al.Significance of ?-alloisoleucine in plasma for diagnosis of maple syrup urine diseaseClin. Chem.45(10)1734-1740(1999) 2.Schadewaldt, P., Bodner-Leidecker, A., Hammen, H.W., et al.Formation of L-alloisoleucine in vivo: An L-[13C]isoleucine study in manPediatr. Res.47(2)271-277(2000)

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1 mg 5 mg 10 mg
1 mM 7.6237 mL 38.1185 mL 76.2369 mL
5 mM 1.5247 mL 7.6237 mL 15.2474 mL
10 mM 0.7624 mL 3.8118 mL 7.6237 mL
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