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The prototypical periplasmic NiFe-H 2 ase (Group 1) [56] consists of a small subunit containing FeS clusters for electron transport (typically a Fe 4 S 4 (SCys) 4 cluster proximal to the NiFe site, a medial Fe 3 S 4 (SCys) 3 cluster, and a distal Fe 4 S 4 (SCys) 3 (His) cluster) and a large protein subunit containing the NiFe active site [59]

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Size:250G

Currently, nine enzymes have been described, the alteration of which can block the production or utilization of Me-Cbl, Ado-Cbl, or both cofactors: lipocalin-1 interacting membrane receptor domain-containing protein 1 (LMBD1), ATP-binding cassette subfamily D member 4 (ABCD4), methylmalonic aciduria type C and homocystinuria (MMACHC), methylmalonic aciduria type D and homocystinuria (MMADHC), methylmalonic aciduria type A (MMAA), ATP-dependent cob(I)alamin adenosyltransferase (ATR), methylmalonyl-CoA mutase (MUT), methionine synthase reductase (MSR) and methionine synthase (MTR) encoded by LMBRD1 , ABCD4 , MMACHC , MMADHC , MMAA , MMAB , MUT , MTRR , and MTR , respectively