IL21

Chr 4AR

interleukin 21

Also known as: CVID11, IL-21, Za11

This gene encodes interleukin-21, a cytokine that regulates both innate and adaptive immune responses by inducing differentiation, proliferation and activity of B cells, T cells, natural killer cells, and macrophages, and is critical for antibody production and T follicular helper cell formation. Mutations cause autosomal recessive common variable immunodeficiency-11, affecting the immune system's ability to produce effective antibody responses. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.643), suggesting some tolerance for reduced function.

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

?Immunodeficiency, common variable, 11MIM #615767
AR
12
Active trials
31
Pubs (1 yr)
23
P/LP submissions
4%
P/LP missense
0.64
LOEUF
Mechanism
Clinical SummaryIL21
🧬
Gene-Disease Validity (ClinGen)
IL21-related infantile inflammatory bowel disease · ARLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.63) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
23 unique Pathogenic / Likely Pathogenic· 76 VUS of 152 total submissions
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Clinical Trials
12 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
0.64LOEUF
pLI 0.625
Z-score 2.18
OE 0.14 (0.050.64)
Moderately constrained

Typical tolerance to LoF variation

Missense Constraint
1.54Z-score
OE missense 0.53 (0.410.68)
44 obs / 83.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.14 (0.050.64)
00.351.4
Missense OE0.53 (0.410.68)
00.61.4
Synonymous OE0.39
01.21.6
LoF obs/exp: 1 / 7.4Missense obs/exp: 44 / 83.6Syn Z: 2.56

ClinVar Variant Classifications

152 submitted variants in ClinVar

Classification Summary

Pathogenic22
Likely Pathogenic1
VUS76
Likely Benign44
Benign8
Conflicting1
22
Pathogenic
1
Likely Pathogenic
76
VUS
44
Likely Benign
8
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
1
21
0
22
Likely Pathogenic
0
0
1
0
1
VUS
3
66
6
1
76
Likely Benign
0
1
30
13
44
Benign
0
0
6
2
8
Conflicting
1
Total3686416152

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

View in ClinVar →

Protein Context — Lollipop Plot

IL21 · 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

HepatoblastomaHepatocellular CarcinomaWilms Tumor

Immunotherapy For Adults With GPC3-Positive Solid Tumors Using IL-15 and IL-21 Armored GPC3-CAR T Cells

RECRUITING
NCT06198296Phase PHASE1Baylor College of MedicineStarted 2025-06-10
21.15.GPC3-CAR T cells
X-linked Severe Combined Immunodeficiency (XSCID)

Lentiviral Gene Transfer for Treatment of Children Older Than Two Years of Age With X-Linked Severe Combined Immunodeficiency (XSCID)

RECRUITING
NCT01306019Phase PHASE1, PHASE2National Institute of Allergy and Infectious Diseases (NIAID)Started 2012-09-25
Ex vivo culture and transduction of the patient's autologous CD34+ HSC with lentivirus vector VSV-G pseudotyped CL20- 4i-EF1alpha-hgammac-OPT vectorBusulfanPalifermin
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
ALS (Amyotrophic Lateral Sclerosis)FTDNeuropathic

Disease Biosignatures in ALS/FTD Spectrum: New Impactful Biological Perspectives Beyond Clinical Approaches

NOT YET RECRUITING
NCT06856850Fondazione I.R.C.C.S. Istituto Neurologico Carlo BestaStarted 2025-06
Solid Tumor (Excluding CNS)Hepatocellular CarcinomaLiver Cell Carcinoma

Cytokine Armored GPC3 Specific Chimeric Antigen Receptor Expressing T-cells in Adults With Solid Tumors

NOT YET RECRUITING
NCT07224568Phase PHASE1Seattle Children's HospitalStarted 2026-04-01
SC-CAR.GPC3xIL15.21 CAR T cells
Hand OsteoarthritisErosive Osteoarthritis

Methotrexate in Erosive Inflammatory Hand Osteoarthritis

ACTIVE NOT RECRUITING
NCT04579848Phase PHASE4Diakonhjemmet HospitalStarted 2021-08-12
Methotrexate TabletsPlaceboFolic Acid 1 MG
Migraine DisordersPainAutoimmune Diseases

The Role of Cytokines and Regulatory T Lymphocytes in Migraine Pathophysiology.

RECRUITING
NCT06426316Phase NAUniversity Hospital, Clermont-FerrandStarted 2025-06-02
Blood test
Advanced Solid Tumors

Safety of Recombinant Human IL-21-expressing Oncolytic Vaccinia Virus Injection (hV01) in Advanced Tumors

ACTIVE NOT RECRUITING
NCT05914376Phase PHASE1Hangzhou Converd Co., Ltd.Started 2023-07-05
Recombinant human IL-21-expressing oncolytic vaccinia virus injection
Liver CancerRhabdomyosarcomaMalignant Rhabdoid Tumor

Interleukin-15 and -21 Armored Glypican-3-specific Chimeric Antigen Receptor Expressed in T Cells for Pediatric Solid Tumors

RECRUITING
NCT04715191Phase PHASE1Baylor College of MedicineStarted 2024-05-24
CARE T cellsCytoxanFludara
Acute-On-Chronic Liver FailureAlcoholic HepatitisLiver Cirrhosis, Alcoholic

A-TANGO Phase 2 Study

NOT YET RECRUITING
NCT06890039Phase PHASE2Yaqrit LtdStarted 2025-09-01
PlaceboTAK-242G-CSF (Filgrastim)
Advanced Solid Tumors

A Study to Evaluate Combination Therapy of Oncolytic Vaccinia Virus (hV01) and PD-1 Inhibitor in Advanced Solid Tumors.

RECRUITING
NCT07185243Phase NAHangzhou Converd Co., Ltd.Started 2025-06-23
recombinant human IL-21-expressing oncolytic vaccinia virus injection
MelanomaMelanoma, UvealHead and Neck Cancer

MAGE-C2 TCR T Cell Trial to Treat Melanoma and Head and Neck Cancer

RECRUITING
NCT04729543Phase PHASE1, PHASE2Erasmus Medical CenterStarted 2020-10-20
Adoptive therapy with autologous MC2 TCR T cells
Clinical Literature
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