CD4

Chr 12AR

CD4 molecule

Also known as: CD4mut, IMD79, Leu-3, OKT4D, T4

The CD4 protein functions as a membrane glycoprotein coreceptor that works with the T-cell receptor to recognize antigens presented by MHC class II molecules, playing an essential role in T-helper cell activation and immune responses. Mutations cause autosomal recessive immunodeficiency disorders including Immunodeficiency 79 and OKT4 epitope deficiency, affecting the immune system's ability to mount appropriate responses to pathogens. The gene shows low constraint against loss-of-function variants (pLI 0.0008), consistent with recessive inheritance where one functional copy provides sufficient protein function.

Summary from RefSeq, OMIM, UniProt
Research Assistant →

Primary Disease Associations & Inheritance

Immunodeficiency 79MIM #619238
AR
OKT4 epitope deficiencyMIM #613949
12
Active trials
9814
Pubs (1 yr)
47
P/LP submissions
2%
P/LP missense
0.71
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryCD4
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
47 unique Pathogenic / Likely Pathogenic· 29 VUS of 120 total submissions
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Clinical Trials
12 active or recruiting trials — potential therapeutic options may be available
📖
GeneReview available — CD4
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
0.71LOEUF
pLI 0.001
Z-score 2.59
OE 0.41 (0.240.71)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.06Z-score
OE missense 0.81 (0.720.91)
206 obs / 253.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.41 (0.240.71)
00.351.4
Missense OE0.81 (0.720.91)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 9 / 22.1Missense obs/exp: 206 / 253.3Syn Z: 0.34
DN
0.77top 25%
GOF
0.6638th %ile
LOF
0.3163th %ile

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

DNprediction above median
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

120 submitted variants in ClinVar

Classification Summary

Pathogenic45
Likely Pathogenic2
VUS29
Likely Benign9
Benign17
45
Pathogenic
2
Likely Pathogenic
29
VUS
9
Likely Benign
17
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
1
1
43
0
45
Likely Pathogenic
0
0
2
0
2
VUS
0
18
11
0
29
Likely Benign
0
1
0
8
9
Benign
0
4
8
5
17
Total1246413102

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

View in ClinVar →

Protein Context — Lollipop Plot

CD4 · 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

Acute Graft-versus-host DiseaseAcute GVHDChronic Graft-versus-host Disease

Dendritic Cells in Patients With Acute or Chronic Skin Graft Versus Host Disease

ACTIVE NOT RECRUITING
NCT02611180Washington University School of MedicineStarted 2015-04-30
Skin punch biopsyPeripheral blood draw
Head and Neck CancerOvarian CancerCervical Cancer

Deciphering Mechanisms Underlying Cancer Immunogenicity

RECRUITING
NCT03958240Phase NAInstitut Claudius RegaudStarted 2020-01-17
Blood samples, tumor biopsy specimens and ascites samples will be collected.
Head and Neck Squamous Cell Carcinoma (HNSCC)

Study of Safety and Tolerability of Nivolumab Treatment Alone or in Combination With Relatlimab or Ipilimumab in Head and Neck Cancer

RECRUITING
NCT04080804Phase PHASE2Dan ZandbergStarted 2019-12-20
NivolumabRelatlimabIpilimumab
HIV (Human Immunodeficiency Virus)HIV -1 Infection

Combining Latency Reversing Agents to Address the HIV Reservoir

NOT YET RECRUITING
NCT07384624Phase PHASE1, PHASE2Erasmus Medical CenterStarted 2026-04
Pyrimethamine (PYR)LenalidomideTopiramate (drug)
Melanoma

Adoptive T Cell Immunotherapy for Advanced Melanoma Using Engineered Lymphocytes

RECRUITING
NCT02870244Phase PHASE1Loyola UniversityStarted 2015-02
Escalating Doses
Hiv

CD4 CAR+ ZFN-modified T Cells in HIV Therapy

ACTIVE NOT RECRUITING
NCT03617198Phase PHASE1University of PennsylvaniaStarted 2019-07-31
CD4 CAR+CCR5 ZFN T-cells
Stable COPD Patients

Study on the Mechanism of Yifei Moxibustion Improving Immune Function in COPD Based on Plasma Exosome miRNAs

RECRUITING
NCT07131020Phase NAHenan University of Traditional Chinese MedicineStarted 2024-05-11
Yifei moxibustionConventional Western medicine treatmentTCM syndrome differentiation treatment
IgG4-Related Diseases

Key Mechanisms of Abnormal T Cell Activation and Differentiation in IgG4-Related Ophthalmic Disease

NOT YET RECRUITING
NCT06655831Peking University Third HospitalStarted 2024-11-01
Lung Non-Small Cell CarcinomaStage IV Lung Cancer AJCC v8

A Multiple Antigen Vaccine (STEMVAC) for the Treatment of Patients With Stage IV Non-Small Cell Lung Cancer

ACTIVE NOT RECRUITING
NCT05242965Phase PHASE2University of WashingtonStarted 2023-03-24
CD105/Yb-1/SOX2/CDH3/MDM2-polyepitope Plasmid DNA VaccineSargramostimComputed Tomography
Lung Non-Small Cell CarcinomaStage IV Lung Cancer AJCC v8Stage IVA Lung Cancer AJCC v8

CCL21-Gene Modified Dendritic Cell Vaccine and Pembrolizumab in Treating Patients With Stage IV Non-small Cell Lung Cancer

ACTIVE NOT RECRUITING
NCT03546361Phase PHASE1Jonsson Comprehensive Cancer CenterStarted 2019-07-08
Autologous Dendritic Cell-Adenovirus CCL21 VaccinePembrolizumab
Advanced Pancreatic Cancer

Recombinant Human IL-21-expressing Oncolytic Vaccinia Virus Injection (hV01) in Advanced Pancreatic Cancer

RECRUITING
NCT07006077Phase PHASE2Hangzhou Converd Co., Ltd.Started 2025-05-29
Recombinant human IL-21-expressing oncolytic vaccinia virus injection
Stroke, AcuteNeuroprotectionIschemic Conditioning

Effectiveness and Biological Mechanism of Direct Ischemic Post-conditioning for Acute Stroke Patients Due to Large Vessel Occlusion

RECRUITING
NCT06545734Phase PHASE2Tianjin Huanhu HospitalStarted 2023-02-05
Direct Ischemic Post-conditioningEndovascular therapy
Clinical Literature
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