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Commission regulation (EU) No 1129/2011
ABCD1 is located in the mitochondrial membrane and is involved in the -oxidation of very long-chain fatty acids (VLCFA), and its deficiency leads to the accumulation of VLCFA, activation of the NLRP3 inflammasome, and release of pro-inflammatory factors such as IL-1 [165]
This enzyme has two catalytic activities (Takahashi, 2011)
