3-Methylcrotonyl CoA

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3-Methylcrotonyl CoA is a key metabolic intermediate in the leucine degradation pathway, converted to 3-Methylglutaconyl CoA by 3-Methylcrotonyl CoA Carboxylase. Normally, 3-Methylglutaconyl CoA is catalyzed by 3-Methylglutaconyl-CoA Hydratase into 3-Hydroxy-3-methylglutaryl-CoA (HMG CoA). However, mutations in 3-Methylglutaconyl-CoA Hydratase lead to the accumulation of 3-Methylglutaconyl CoA, causing metabolic disorders associated with 3MGA-uria. 3-Methylcrotonyl CoA is useful for research into metabolic diseases related to the leucine degradation pathway.

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3-Methylcrotonyl CoA Chemical Structure

CAS No. : 6712-03-4

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Description	3-Methylcrotonyl CoA is a key metabolic intermediate in the leucine degradation pathway, converted to 3-Methylglutaconyl CoA by 3-Methylcrotonyl CoA Carboxylase. Normally, 3-Methylglutaconyl CoA is catalyzed by 3-Methylglutaconyl-CoA Hydratase into 3-Hydroxy-3-methylglutaryl-CoA (HMG CoA). However, mutations in 3-Methylglutaconyl-CoA Hydratase lead to the accumulation of 3-Methylglutaconyl CoA, causing metabolic disorders associated with 3MGA-uria. 3-Methylcrotonyl CoA is useful for research into metabolic diseases related to the leucine degradation pathway^[1].
Molecular Weight	849.63
Formula	C₂₆H₄₂N₇O₁₇P₃S
CAS No.	6712-03-4
SMILES	NC1=NC=NC2=C1N=CN2[C@H]3[C@H](O)[C@H](OP(O)(O)=O)[C@@H](COP(OP(OCC(C)(C)[C@@H](O)C(NCCC(NCCSC(/C=C(C)\C)=O)=O)=O)(O)=O)(O)=O)O3
Structure Classification	Others
Initial Source	Endogenous metabolite
Shipping	Room temperature in continental US; may vary elsewhere.
Storage	Please store the product under the recommended conditions in the Certificate of Analysis.
Purity & Documentation	Data Sheet (265 KB) Handling Instructions (2659 KB)
References	[1]. Su B, et al. Metabolic biology of 3-methylglutaconic acid-uria: a new perspective. J Inherit Metab Dis. 2014 May;37(3):359-68. [Content Brief]