SERPINA7

Chr XX-linked

serpin family A member 7

Also known as: TBG, TBGQTL

This gene encodes thyroxine-binding globulin (TBG), the major thyroid hormone transport protein in serum that carries T4 and T3 in the bloodstream. Mutations cause TBG deficiency, which can range from partial to complete deficiency or excess based on serum TBG levels, with X-linked inheritance. The gene shows low constraint to loss-of-function variants (pLI 0.003), consistent with TBG deficiency typically being a biochemical finding rather than causing severe clinical disease.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

[Thyroxine-binding globulin QTL]MIM #300932
X-linked
[Thyroxine-binding globulin QTL]MIM #300932
X-linked
0
Active trials
8
Pubs (1 yr)
0
P/LP submissions
P/LP missense
LOEUF
Multiple*
Mechanism· predicted
Some data sources returned errors (1)

gnomad: TimeoutError: The operation was aborted due to timeout

Population Genetics & Constraint

Constraint data not available from gnomAD.

DN
0.78top 25%
GOF
0.79top 25%
LOF
0.1499th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SERPINA7 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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