The complex machinery of human cobalamin metabolism

TJ McCorvie, D Ferreira, WW Yue… - Journal of Inherited …, 2023 - Wiley Online Library
Abstract Vitamin B12 (cobalamin, Cbl) is required as a cofactor by two human enzymes, 5‐
methyltetrahydrofolate‐homocysteine methyltransferase (MTR) and methylmalonyl‐CoA …

Structure of MMACHC Reveals an Arginine-Rich Pocket and a Domain-Swapped Dimer for Its B12 Processing Function

DS Froese, T Krojer, X Wu, R Shrestha, W Kiyani… - Biochemistry, 2012 - ACS Publications
Defects in the MMACHC gene represent the most common disorder of cobalamin (Cbl)
metabolism, affecting synthesis of the enzyme cofactors adenosyl-Cbl and methyl-Cbl. The …

Structural insights into the MMACHC-MMADHC protein complex involved in vitamin B12 trafficking

DS Froese, J Kopec, F Fitzpatrick, M Schuller… - Journal of Biological …, 2015 - ASBMB
Conversion of vitamin B 12 (cobalamin, Cbl) into the cofactor forms methyl-Cbl (MeCbl) and
adenosyl-Cbl (AdoCbl) is required for the function of two crucial enzymes, mitochondrial …

Navigating the B12 road: assimilation, delivery, and disorders of cobalamin

C Gherasim, M Lofgren, R Banerjee - Journal of Biological Chemistry, 2013 - ASBMB
The reactivity of the cobalt-carbon bond in cobalamins is the key to their chemical versatility,
supporting both methyl transfer and isomerization reactions. During evolution of higher …

Structure of human B12 trafficking protein CblD reveals molecular mimicry and identifies a new subfamily of nitro-FMN reductases

K Yamada, C Gherasim, R Banerjee… - Journal of Biological …, 2015 - ASBMB
In mammals, B 12 (or cobalamin) is an essential cofactor required by methionine synthase
and methylmalonyl-CoA mutase. A complex intracellular pathway supports the assimilation …

Pathogenic mutations differentially affect the catalytic activities of the human B12-processing chaperone CblC and increase futile redox cycling

C Gherasim, M Ruetz, Z Li, S Hudolin… - Journal of Biological …, 2015 - ASBMB
Human CblC catalyzes the elimination of the upper axial ligand in cobalamin or B 12
derivatives entering the cell from circulation. This processing step is critical for assimilation …

The human B12 trafficking protein CblC processes nitrocobalamin

R Mascarenhas, Z Li, C Gherasim, M Ruetz… - Journal of Biological …, 2020 - ASBMB
In humans, cobalamin or vitamin B 12 is delivered to two target enzymes via a complex
intracellular trafficking pathway comprising transporters and chaperones. CblC (or …

Structural basis of multifunctionality in a vitamin B12-processing enzyme

M Koutmos, C Gherasim, JL Smith… - Journal of Biological …, 2011 - ASBMB
An early step in the intracellular processing of vitamin B 12 involves CblC, which exhibits
dual reactivity, catalyzing the reductive decyanation of cyanocobalamin (vitamin B 12), and …

Redox-Linked Coordination Chemistry Directs Vitamin B12 Trafficking

R Banerjee, H Gouda, S Pillay - Accounts of chemical research, 2021 - ACS Publications
Conspectus Metals are partners for an estimated one-third of the proteome and vary in
complexity from mononuclear centers to organometallic cofactors. Vitamin B12 or cobalamin …

Clinical or ATPase domain mutations in ABCD4 disrupt the interaction between the vitamin B12-trafficking proteins ABCD4 and LMBD1

V Fettelschoss, P Burda, C Sagné, D Coelho… - Journal of Biological …, 2017 - ASBMB
Vitamin B 12 (cobalamin (Cbl)), in the cofactor forms methyl-Cbl and adenosyl-Cbl, is
required for the function of the essential enzymes methionine synthase and methylmalonyl …