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Transcobalamin

From Wikipedia, the free encyclopedia
Group of carrier proteins which bind with Vitamin B12 in the blood
transcobalamin I (vitamin B12 binding protein, R binder family)
Identifiers
SymbolTCN1
NCBI gene6947
HGNC11652
OMIM189905
RefSeqNM_001062
UniProtP20061
Other data
LocusChr. 11q11-q12
Search for
StructuresSwiss-model
DomainsInterPro
transcobalamin II; macrocytic anemia
Identifiers
SymbolTCN2
NCBI gene6948
HGNC11653
OMIM275350
PDB2BB5
RefSeqNM_000355
UniProtP20062
Other data
LocusChr. 22q11.2-qter
Search for
StructuresSwiss-model
DomainsInterPro

Transcobalamins arecarrier proteins which bindcobalamin (B12).

Types

[edit]

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-Vitamin B12 complex. Once the complex has traveled to the more pH-neutral duodenum, pancreatic proteases degrade haptocorrin, releasing free cobalamin, which now binds tointrinsic factor for absorption byileal enterocytes.

Separate from the digestive absorption function, serum TC-1 binds 80-90% of circulating B12, 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 vitamin B12.[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 "Intrinsic Factor-Vitamin B12" complex has been degraded. TC-2 is then involved with the transport of Vitamin B12 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]

References

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  1. ^McCorvie TJ, Ferreira D, Yue WW, Froese DS (May 2023). "The complex machinery of human cobalamin metabolism".J Inherit Metab Dis.46 (3):406–20.doi:10.1002/jimd.12593.PMID 36680553.
  2. ^Ermens AA, Vlasveld LT, Lindemans J (November 2003). "Significance of elevated cobalamin (vitamin B12) levels in blood".Clin Biochem.36 (8):585–90.doi:10.1016/j.clinbiochem.2003.08.004.PMID 14636871.
  3. ^Seetharam B, Li N (2000). "Transcobalamin II and its cell surface receptor".Vitam Horm. Vitamins & Hormones.59:337–66.doi:10.1016/s0083-6729(00)59012-8.ISBN 978-0-12-709859-3.PMID 10714245.

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