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Results 1 - 4 of 4

EC Number	Natural Substrates	Commentary (Nat. Sub.)
1.1.1.178	2-methyl-3-hydroxybutyryl-CoA + NAD+	-
1.1.1.178	2-methyl-3-hydroxybutyryl-CoA + NAD+	isoleucine metabolism
1.1.1.178	more	genetic deficiency of enzyme activity leads to neurodegenerative disease, that can be lessened by dietary isoleucine restriction, detection of high urinary levels of 2-methyl-3-hydroxybutyryl-CoA and tiglyl-glycine
1.1.1.178	more	inborn X-linked deficiency in 2-methyl-3-hydroxybutyryl-CoA dehydrogenase, MHBD, causes lethal neurodegenerative disease due to an error in isoleucine metabolism, overview

Results 1 - 4 of 4

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Release 2023.1 (January 2023)