| transcobalamin I (vitamin B12 binding protein, R binder family) | |||||||
|---|---|---|---|---|---|---|---|
| Identifiers | |||||||
| Symbol | TCN1 | ||||||
| NCBI gene | 6947 | ||||||
| HGNC | 11652 | ||||||
| OMIM | 189905 | ||||||
| RefSeq | NM_001062 | ||||||
| UniProt | P20061 | ||||||
| Other data | |||||||
| Locus | Chr. 11q11-q12 | ||||||
| |||||||
| transcobalamin II; macrocytic anemia | |||||||
|---|---|---|---|---|---|---|---|
| Identifiers | |||||||
| Symbol | TCN2 | ||||||
| NCBI gene | 6948 | ||||||
| HGNC | 11653 | ||||||
| OMIM | 275350 | ||||||
| PDB | 2BB5 | ||||||
| RefSeq | NM_000355 | ||||||
| UniProt | P20062 | ||||||
| Other data | |||||||
| Locus | Chr. 22q11.2-qter | ||||||
| |||||||
Transcobalamins arecarrier proteins which bindcobalamin (vitamin B12).
Transcobalamin I (TC-1), also known ashaptocorrin (HC),R-factor, andR-protein is encoded in the human by theTCN1 gene. TC-1 is a glycoprotein produced by thesalivary glands of the mouth. It primarily serves to protectcobalamin (vitamin B12) from acid degradation in the stomach by producing a HC-cobalamin complex. Once the complex has traveled to the more pH-neutral duodenum, pancreatic proteases degrade haptocorrin, releasing free cobalamin, which now binds tointrinsic factor (IF) for absorption byileal enterocytes.
Separate from the digestive absorption function, serum TC-1 binds 80–90% of circulating cobalamin, rendering it unavailable for cellular delivery by TC-2.[1] Several serious, even life-threatening diseases cause elevated serum HC, measured as abnormally high serum cobalamin.[2]
Transcobalamin II (TC-2), a nonglycoprotein secretory protein of molecular mass 43 kDa, is encoded in the human by theTCN2 gene. TC-2 binds cobalamin once it has been taken up byenterocytes of the terminal ileum and the IF-cobalamin complex has been degraded. TC-2 is then involved with the transport of cobalamin to the tissues, where it binds to its plasma membrane receptor (TC-2R), a heavily glycosylated protein with a monomeric molecular mass of 62 kDa, and releases cobalamin to the cells.[3]