SEC61B

Chr 9

SEC61 translocon subunit beta

SEC61B encodes the beta subunit of the SEC61 translocon complex, which forms channels in the endoplasmic reticulum membrane that transport signal peptide-containing proteins across the ER membrane and insert transmembrane proteins into the ER membrane. Pathogenic variants in SEC61B have not been established as a cause of human disease. The gene shows tolerance to loss-of-function variants (pLI = 0.02, LOEUF = 1.48), suggesting haploinsufficiency is unlikely to be pathogenic.

Summary from RefSeq, UniProt, Mechanism
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0
Active trials
6
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.48
LOEUF
DN
Mechanism· predicted
Clinical SummarySEC61B
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Gene-Disease Validity (ClinGen)
SEC61B-related polycystic liver disease · ADLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Low constraint (pLI 0.02) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.48LOEUF
pLI 0.020
Z-score 0.85
OE 0.59 (0.271.48)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.03Z-score
OE missense 0.61 (0.470.81)
35 obs / 56.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.59 (0.271.48)
00.351.4
Missense OE0.61 (0.470.81)
00.61.4
Synonymous OE1.31
01.21.6
LoF obs/exp: 3 / 5.1Missense obs/exp: 35 / 56.9Syn Z: -1.15
DN
0.78top 25%
GOF
0.4184th %ile
LOF
0.3454th %ile

The highest-scoring mechanism for this gene is dominant-negative.

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

SEC61B · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC