SOCS1

Chr 16AD

suppressor of cytokine signaling 1

Also known as: AISIMD, CIS1, CISH1, JAB, SOCS-1, SSI-1, SSI1, TIP-3

The protein functions as an essential negative regulator of interferon and cytokine signaling by inhibiting the JAK/STAT pathway and promoting degradation of target proteins through its role in an E3 ubiquitin ligase complex. Mutations cause autosomal dominant autoinflammatory syndrome, familial, with or without immunodeficiency, affecting the immune system through dysregulated cytokine responses. The gene shows moderate constraint against loss-of-function variants (LOEUF 1.068), consistent with its role in immune regulation.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismADLOEUF 1.071 OMIM phenotype
Clinical SummarySOCS1
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Gene-Disease Validity (ClinGen)
autoinflammatory syndrome with immunodeficiency · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.59) — some intolerance to loss-of-function variants.
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Clinical Trials
3 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
1.07LOEUF
pLI 0.585
Z-score 1.53
OE 0.00 (0.001.07)
Moderately constrained

Highly tolerant — LoF variants common in population

Missense Constraint
0.83Z-score
OE missense 0.75 (0.620.92)
67 obs / 89.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.00 (0.001.07)
00.351.4
Missense OE0.75 (0.620.92)
00.61.4
Synonymous OE1.61
01.21.6
LoF obs/exp: 0 / 2.7Missense obs/exp: 67 / 89.0Syn Z: -3.09
DN
0.4784th %ile
GOF
0.5955th %ile
LOF
0.56top 25%

The Badonyi & Marsh model scores gain-of-function highest, but genomic evidence most strongly supports loss-of-function (haploinsufficiency) as the primary mechanism.

LOF1 literature citation

Literature Evidence

LOFImmune dysregulation and multisystem inflammatory syndrome in children (MIS-C) in individuals with haploinsufficiency of SOCS1.PMID:32853638

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SOCS1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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