SECISBP2

Chr 9AR

SECIS binding protein 2

The protein binds to SECIS elements in the 3'-UTR of selenoprotein mRNAs and facilitates incorporation of selenocysteine at UGA codons during translation. Autosomal recessive mutations cause abnormal thyroid hormone metabolism type 1 through reduced activity of selenoproteins, particularly type II iodothyronine deiodinase. The pathogenic mechanism involves impaired selenocysteine insertion into selenoproteins, disrupting their enzymatic function.

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

Thyroid hormone metabolism, abnormal, 1MIM #609698
AR
0
Active trials
5
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.03
LOEUF
LOF
Mechanism· G2P
Clinical SummarySECISBP2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.03LOEUF
pLI 0.000
Z-score 1.41
OE 0.77 (0.581.03)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.01Z-score
OE missense 1.00 (0.921.08)
439 obs / 439.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.77 (0.581.03)
00.351.4
Missense OE1.00 (0.921.08)
00.61.4
Synonymous OE1.22
01.21.6
LoF obs/exp: 33 / 42.9Missense obs/exp: 439 / 439.3Syn Z: -2.23

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SECISBP2 · protein map & ClinVar variants

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

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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