TCII:Cbl is degraded to release Cbl

Stable Identifier
R-HSA-3000263
Type
Reaction [omitted]
Species
Homo sapiens
Compartment
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Once in the lysosome, transcobalamin 2:cobalamin (TCII:Cbl) is degraded to release Cbl (Youngdahl-Turner et al. 1979). Cbl is ready to be exported out of the lysosome to the cytosol by the probable lysosomal cobalamin transporter (LMBRD1) (Rutsch et al. 2009). Once in the cytosol, Cbl can be used in the synthesis of the essential cofactors methylcobalamin (MeCbl) and adenosylcobalamin (AdoCbl) as described in the steps above.

Literature References
PubMed ID Title Journal Year
19136951 Identification of a putative lysosomal cobalamin exporter altered in the cblF defect of vitamin B12 metabolism

Rutsch, F, Gailus, S, Miousse, IR, Suormala, T, Sagné, C, Toliat, MR, Nürnberg, G, Wittkampf, T, Buers, I, Sharifi, A, Stucki, M, Becker, C, Baumgartner, M, Robenek, H, Marquardt, T, Höhne, W, Gasnier, B, Rosenblatt, DS, Fowler, B, Nürnberg, P

Nat. Genet. 2009
759210 Protein mediated vitamin uptake. Adsorptive endocytosis of the transcobalamin II-cobalamin complex by cultured human fibroblasts

Youngdahl-Turner, P, Mellman, IS, Allen, RH, Rosenberg, LE

Exp. Cell Res. 1979
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