The human B₁₂ trafficking protein CblC processes nitrocobalamin.

ABSTRACT: In humans, cobalamin or vitamin B₁₂ is delivered to two target enzymes via a complex intracellular trafficking pathway comprising transporters and chaperones. CblC (or MMACHC) is a processing chaperone that catalyzes an early step in this trafficking pathway. CblC removes the upper axial ligand of cobalamin derivatives, forming an intermediate in the pathway that is subsequently converted to the active cofactor derivatives. Mutations in the cblC gene lead to methylmalonic aciduria and homocystinuria. Here, we report that nitrosylcobalamin (NOCbl), which was developed as an antiproliferative reagent, and is purported to cause cell death by virtue of releasing nitric oxide, is highly unstable in air and is rapidly oxidized to nitrocobalamin (NO₂Cbl). We demonstra

SUBMITTER: Mascarenhas R 

PROVIDER: S-EPMC7363124 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature