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. 2016 Jun 11;32(5):733–741. doi: 10.1007/s00467-016-3399-0

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© The Author(s) 2016

Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

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Fig. 1 — Methylmalonic aciduria and homocystinuria, cobalamin C (cblC) complementation type (MMACHC) is required for decyanation of cyanocobalamin, a precursor for subsequent conversion into the essential cofactors methylcobalamin (MeCbl) and adenosylcobalamin (AdoCbl). MeCbl is required to metabolize homocysteine to methionine, and AdoCbl is needed for the breakdown of methylmalonic acid. This scheme illustrates why cyanocobalamin is largely ineffective to treat cblC defect and why blood levels of both homocysteine and methylmalonic acid are increased while methionine concentrations are reduced (modified from [7], used with permission)