规格 | 10mg 50mg 100mg 250mg |
CAS | 33008-07-0 |
别名 | N/A |
化学名 | N-(3-methyl-1-oxo-2-buten-1-yl)-glycine |
分子式 | C7H11NO3 |
分子量 | 157.2 |
溶解度 | 50mg/mL in ethanol, 20mg/mL in DMSO |
储存条件 | Store at -20°C |
General tips | For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while. |
Shipping Condition | Evaluation sample solution : ship with blue ice |
产品描述:
3-Methylcrotonyl glycine (3-MCG) is a metabolite found in the urine of patients with 3-methylcrotonyl glycinuria, a metabolic disorder characterized by a deficiency in 3-methylcrotonyl-CoA carboxylase, that has diverse biological activities.[1] [2] It inhibits CO2 production and mitochondrial complex II-III and creatine kinase activity in rat cerebral cortex preparations in a concentration-dependent manner.[2] It also inhibits the Na+/K+-ATPase in purified synaptic membranes from rat cerebrum, indicating a potential role for this metabolite in the development of CNS damage associated with 3-methycrotonyl glycinuria.
Reference:
[1]. Bartlett, K., Ng, H., and Leonard, J.V. A combined defect of three mitochondrial carboxylases presenting as biotin-responsive 3-methylcrotonyl glycinuria and 3-hydroxyisovaleric aciduria. Clin. Chim. Acta. 100(2), 183-186 (1980).
[2]. Moura, A.P., Ribeiro, C.A., Zanatta, ?., et al. 3-Methylcrotonylglycine disrupts mitochondrial energy homeostasis and inhibits synaptic Na+,K+-ATPase activity in brain of young rats. Cell. Mol. Neurobiol. 32(2), 297-307 (2012).
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