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10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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TCN2
transcobalamin 2
Chromosome 22 Β· 22q12.2
NCBI Gene: 6948Ensembl: ENSG00000185339.11HGNC: HGNC:11653UniProt: P20062
146PubMed Papers
21Diseases
0Drugs
70Pathogenic Variants
FUNCTIONAL ROLE
Transporter
RESEARCH IMPACT
Variant-Rich
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
protein bindingcobalamin bindingcargo receptor ligand activitycobalamin transporttranscobalamin II deficiencyTranscobalamin deficiencyPancytopeniaagammaglobulinemia
✦AI Summary

TCN2 encodes transcobalamin 2 (TC), the primary vitamin B12-binding and transport protein essential for delivering cobalamin to cells 1. TC binds cobalamin with high affinity through specific amino acid residues and facilitates cellular uptake via receptor-mediated endocytosis, with the binding interaction structurally conserved across variants 2. TCN2 deficiency causes severe early-onset disease presenting in infancy with failure to thrive, pancytopenia, hypogammaglobulinemia, and neurological manifestations despite normal circulating B12 levels, reflecting impaired cellular cobalamin delivery 1. Beyond classical deficiency, emerging evidence suggests TCN2 variants contribute to complex diseases: a missense mutation (p.K77M) associated with diabetic nephropathy induces mitochondrial dysfunction and renal tubular damage 3, while TCN2 was identified as a novel susceptibility locus for Alzheimer's disease in Chinese populations 4. Interestingly, TCN2 exhibits unexpected antiviral properties, with upregulation following respiratory syncytial virus infection and demonstrated capacity to inhibit viral replication, suggesting roles beyond canonical B12 transport 5. TCN2 polymorphisms show population-specific effects on B12 metabolism and hematological parameters 6, though associations with nonsyndromic cleft palate remain inconsistent across ethnic groups 78. These findings indicate TCN2 functions extend beyond vitamin B12 transport to encompass immune regulation, metabolic homeostasis, and cellular stress responses.

Sources cited
1
TCN2 deficiency causes early-onset disease with failure to thrive, pancytopenia, hypogammaglobulinemia, and impaired cellular cobalamin delivery
PMID: 32841161
2
Structural characterization of TCN2 B12-binding interactions and effects of missense mutations on binding affinity
PMID: 35261335
3
TCN2 p.K77M variant identified as top candidate locus for diabetic nephropathy, inducing mitochondrial dysfunction and renal tubular damage
PMID: 40712574
4
TCN2 identified as novel AD susceptibility locus in Chinese populations through genome-wide association study
PMID: 39023044
5
TCN2 exhibits antiviral properties, upregulated following RSV infection and inhibits viral replication
PMID: 39313785
6
TCN2 polymorphisms influence circulating TCN2 levels and hematological parameters in B12 deficiency
PMID: 41175296
7
TCN2 C776G polymorphism shows no significant association with nonsyndromic cleft lip/palate risk
PMID: 30004262
8
TCN2 genetic variations not associated with nonsyndromic cleft lip/palate in Chinese population
PMID: 25105440
Disease Associationsβ“˜21
transcobalamin II deficiencyOpen Targets
0.81Strong
Transcobalamin deficiencyOpen Targets
0.79Strong
PancytopeniaOpen Targets
0.63Moderate
agammaglobulinemiaOpen Targets
0.54Moderate
vitamin B12 deficiencyOpen Targets
0.51Moderate
megaloblastic anemiaOpen Targets
0.51Moderate
Failure to thriveOpen Targets
0.51Moderate
Decreased total neutrophil countOpen Targets
0.50Moderate
neutropeniaOpen Targets
0.50Moderate
ThrombocytopeniaOpen Targets
0.50Moderate
vitamin B deficiencyOpen Targets
0.47Moderate
Intellectual disabilityOpen Targets
0.46Moderate
Absence of circulating granulocytesOpen Targets
0.46Moderate
severe combined immunodeficiencyOpen Targets
0.46Moderate
genetic disorderOpen Targets
0.42Moderate
deficiency anemiaOpen Targets
0.41Moderate
vitamin deficiency disorderOpen Targets
0.41Moderate
GrΓ€sbeck-Imerslund diseaseOpen Targets
0.37Weak
transcobalamin I deficiencyOpen Targets
0.37Weak
Alzheimer diseaseOpen Targets
0.33Weak
Transcobalamin II deficiencyUniProt
Pathogenic Variants70
NM_000355.4(TCN2):c.65-1_65delinsTTLikely pathogenic
Transcobalamin II deficiency|not provided
β˜…β˜…β˜†β˜†2026
NM_000355.4(TCN2):c.562C>T (p.Gln188Ter)Pathogenic
not provided|Inborn genetic diseases|Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 188
NM_000355.4(TCN2):c.1099G>T (p.Gly367Ter)Pathogenic
not provided|Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2025β†’ Residue 367
NM_000355.4(TCN2):c.428-2_428-1delPathogenic
Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2025
NM_000355.4(TCN2):c.679C>T (p.Arg227Ter)Pathogenic
Transcobalamin II deficiency|TCN2-related disorder
β˜…β˜…β˜†β˜†2025β†’ Residue 227
NM_000355.4(TCN2):c.1127dup (p.Leu376fs)Pathogenic
not provided|Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2024β†’ Residue 376
NM_000355.4(TCN2):c.871C>T (p.Gln291Ter)Pathogenic
not provided|Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2024β†’ Residue 291
NM_000355.4(TCN2):c.350_351insAC (p.Phe118fs)Pathogenic
Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2024β†’ Residue 118
NM_000355.4(TCN2):c.38del (p.Val13fs)Pathogenic
Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2024β†’ Residue 13
NM_000355.4(TCN2):c.997dup (p.Thr333fs)Pathogenic
not provided|Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2024β†’ Residue 333
NM_000355.4(TCN2):c.344del (p.Asn115fs)Pathogenic
TCN2-related disorder|Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2023β†’ Residue 115
NM_000355.4(TCN2):c.428-2A>GPathogenic
not provided|Transcobalamin II deficiency|Pancytopenia
β˜…β˜…β˜†β˜†2023
NM_000355.4(TCN2):c.1106+1G>APathogenic
Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2021
NM_000355.4(TCN2):c.766dup (p.Ser256fs)Pathogenic
Transcobalamin II deficiency
β˜…β˜…β˜†β˜†2020β†’ Residue 256
NM_000355.4(TCN2):c.244C>T (p.Gln82Ter)Pathogenic
Transcobalamin II deficiency
β˜…β˜†β˜†β˜†2026β†’ Residue 82
NM_000355.4(TCN2):c.821del (p.Leu274fs)Pathogenic
Transcobalamin II deficiency
β˜…β˜†β˜†β˜†2026β†’ Residue 274
NM_000355.4(TCN2):c.258-1G>TLikely pathogenic
Transcobalamin II deficiency
β˜…β˜†β˜†β˜†2025
NM_000355.4(TCN2):c.116dup (p.Leu39fs)Pathogenic
Transcobalamin II deficiency
β˜…β˜†β˜†β˜†2025β†’ Residue 39
NM_000355.4(TCN2):c.836del (p.Gln279fs)Pathogenic
Transcobalamin II deficiency
β˜…β˜†β˜†β˜†2025β†’ Residue 279
NM_000355.4(TCN2):c.271G>T (p.Glu91Ter)Pathogenic
Transcobalamin II deficiency
β˜…β˜†β˜†β˜†2025β†’ Residue 91
View on ClinVar β†—
Related Genes
CBLIFShared pathway100%TCN1Shared pathway100%CBLProtein interaction92%LTFProtein interaction84%LMBRD1Protein interaction83%MMUTProtein interaction81%
Tissue Expression6 tissues
Lung
100%
Heart
57%
Ovary
45%
Brain
40%
Liver
29%
Bone Marrow
26%
Gene Interaction Network
Click a node to explore
TCN2CBLIFTCN1CBLLTFLMBRD1MMUT
PROTEIN STRUCTURE
Preparing viewer…
PDB5NSA Β· 1.27 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.89LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.67 [0.51–0.89]
RankingsWhere TCN2 stands among ~20K protein-coding genes
  • #3,121of 20,598
    Most Researched146 Β· top quartile
  • #1,038of 5,498
    Most Pathogenic Variants70 Β· top quartile
  • #7,936of 17,882
    Most Constrained (LOEUF)0.89
Genes detectedTCN2
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Innovative biomarkers TCN2 and LY6E can significantly inhibit respiratory syncytial virus infection.
PMID: 39313785
J Transl Med Β· 2024
1.00
2
Genome-wide meta-analysis identifies ancestry-specific loci for Alzheimer's disease.
PMID: 39023044
Alzheimers Dement Β· 2024
0.90
3
Associations Between MTR A2756G, MTRR A66G, and TCN2 C776G Polymorphisms and Risk of Nonsyndromic Cleft Lip With or Without Cleft Palate: A Meta-Analysis.
PMID: 30004262
Genet Test Mol Biomarkers Β· 2018
0.80
4
Vitamin B12 binding to mutated human transcobalamin, in-silico study of TCN2 alanine scanning and ClinVar missense mutations/SNPs.
PMID: 35261335
J Biomol Struct Dyn Β· 2023
0.70
5
Multimodal analysis stratifies genetic susceptibility and reveals the pathogenic mechanism of kidney injury in diabetic nephropathy.
PMID: 40712574
Cell Rep Med Β· 2025
0.60