MYD88

Chr 3AR

MYD88 innate immune signal transduction adaptor

Also known as: IMD68, MYD88D, WM1

The protein functions as a cytosolic adapter protein essential for Toll-like receptor and IL-1 receptor signaling pathways in innate and adaptive immune responses, leading to NF-kappa-B activation and cytokine secretion. Autosomal recessive mutations cause Immunodeficiency 68, characterized by increased susceptibility to pyogenic bacterial infections. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.655), consistent with its role in immune system function.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
GOFmechanismARLOEUF 0.662 OMIM phenotypes
Clinical SummaryMYD88
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.29) despite low pLI — interpret in context.
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ClinVar Variants
17 unique Pathogenic / Likely Pathogenic· 92 VUS of 211 total submissions
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Clinical Trials
9 active or recruiting trials — potential therapeutic options may be available
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GeneReview available — MYD88
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.66LOEUF
pLI 0.124
Z-score 2.47
OE 0.29 (0.140.66)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.11Z-score
OE missense 0.77 (0.670.89)
143 obs / 185.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.29 (0.140.66)
00.351.4
Missense OE0.77 (0.670.89)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 4 / 14.0Missense obs/exp: 143 / 185.7Syn Z: -0.56
DN
0.6163th %ile
GOF
0.77top 25%
LOF
0.2774th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction 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

211 submitted variants in ClinVar

Classification Summary

Pathogenic14
Likely Pathogenic3
VUS92
Likely Benign84
Benign7
Conflicting2
14
Pathogenic
3
Likely Pathogenic
92
VUS
84
Likely Benign
7
Benign
2
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
2
1
11
0
14
Likely Pathogenic
1
1
1
0
3
VUS
4
70
18
0
92
Likely Benign
0
10
28
46
84
Benign
0
3
1
3
7
Conflicting
2
Total7855949202

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

MYD88 · 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

Waldenstrom MacroglobulinemiaMYD88 Gene Mutation

Ibrutinib + Venetoclax in Untreated WM

ACTIVE NOT RECRUITING
NCT04273139Phase PHASE2Dana-Farber Cancer InstituteStarted 2020-07-09
IBRUTINIBVenetoclax
Recurrent Diffuse Large B-Cell Lymphoma Activated B-Cell TypeRefractory Diffuse Large B-Cell Lymphoma Activated B-Cell Type

Ibrutinib Before and After Stem Cell Transplant in Treating Patients With Relapsed or Refractory Diffuse Large B-cell Lymphoma

ACTIVE NOT RECRUITING
NCT02443077Phase PHASE3National Cancer Institute (NCI)Started 2016-10-12
Autologous Bone Marrow TransplantationAutologous Hematopoietic Stem Cell TransplantationCarmustine
Relapsed/Refractory Diffuse Large B-Cell Lymphoma

A Phase III Study of HMPL-760 Plus R-GemOx VS Placebo Plus R-GemOx in Relapsed/Refractory DLBCL

RECRUITING
NCT07409428Phase PHASE3HutchmedStarted 2026-03-20
HMPL-760HMPL-760 PlaceboR-GemOx
Waldenstrom's DiseaseWaldenstrom Macroglobulinemia

Prognostic Value of Circulating Tumoral DNA After the First 6 Months of Treatment in Patients With Waldenström Macroglobulinemia

RECRUITING
NCT04893564Phase NACentre Hospitalier Universitaire, AmiensStarted 2022-05-16
bone marrow sampleblood sample
Relapsed/Refractory Diffuse Large B-Cell Lymphoma

Study of HMPL-760 Plus R-GemOx Versus Placebo Plus R-GemOx in Relapsed/Refractory DLBCL

ACTIVE NOT RECRUITING
NCT06601504Phase PHASE2HutchmedStarted 2024-11-05
HMPL-760 planned dose 1R-GemOxHMPL-760 placebo planned dose 1
Waldenström Macroglobulinemia (WM)

Ibrutinib Followed by BR (Bendamustine and Rituximab) as a Time-Limited Therapy for Waldenström Macroglobulinemia

NOT YET RECRUITING
NCT07169565Phase PHASE1Institute of Hematology & Blood Diseases Hospital, ChinaStarted 2025-09-01
IbrutinibBendamustineRituximab
AsthmaAtherosclerosisMetabolic Syndrome

Study of the Effect of Innate on the Inflammatory Response to Endotoxin

RECRUITING
NCT01143480National Institute of Environmental Health Sciences (NIEHS)Started 2012-07-30
Waldenstrom's DiseasePrognostic Index

Prognostic Analyses on a Validation Series of Patients With Waldenström's Disease

RECRUITING
NCT05911802French Innovative Leukemia OrganisationStarted 2023-08-11
Adequate Vitamin C StatusInadequate Vitamin C Status

Anti-inflammatory Activities of Vitamin C Supplementation on the Gut Barrier Function in Adults With Obesity

RECRUITING
NCT07151105Phase NAOhio State UniversityStarted 2025-10-01
Vitamin C Supplement + Low Vitamin C DietPlacebo + Low Vitamin C Diet
Clinical Literature
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