BCL11A

Chr 2AD

BCL11 transcription factor A

Also known as: CTIP1, DILOS, EVI9, HBFQTL5, SMARCM1, ZNF856

This gene encodes a C2H2 type zinc-finger protein by its similarity to the mouse Bcl11a/Evi9 protein. The corresponding mouse gene is a common site of retroviral integration in myeloid leukemia, and may function as a leukemia disease gene, in part, through its interaction with BCL6. During hematopoietic cell differentiation, this gene is down-regulated. It is possibly involved in lymphoma pathogenesis since translocations associated with B-cell malignancies also deregulates its expression. Multiple transcript variants encoding several different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Dias-Logan syndromeMIM #617101
AD
UniProtIntellectual developmental disorder with persistence of fetal hemoglobin

Clinical highlights

Gene-disease validity (ClinGen)
Dias-Logan syndrome · ADDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype) and the gene is intolerant of it in the population — truncating, frameshift and canonical splice variants carry more prior weight here than missense.Curated gene-level mechanism — a prior for triage, not a per-variant call.
11
Active trials
139
Pubs (1 yr)
P/LP submissions
P/LP missense
0.32
LOEUF· LoF intol.
LOF
Mechanism· G2P
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GeneReview available — BCL11A
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint?
0.32LOEUF
pLI 0.970
Z-score 3.98
OE 0.12 (0.060.32)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint?
3.84Z-score
OE missense 0.54 (0.490.59)
296 obs / 548.8 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios?
LoF OE?0.12 (0.060.32)
00.351.4
Missense OE?0.54 (0.490.59)
00.61.4
Synonymous OE?0.83
01.21.6
LoF obs/exp: 3 / 24.1Missense obs/exp: 296 / 548.8Syn Z: 2.10

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BCL11A · 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

Beta-Thalassemia

Safety and Efficacy Evaluation of BRL-101 in Subjects With Transfusion-Dependent β-Thalassemia

ACTIVE NOT RECRUITING
NCT05577312Phase PHASE1, PHASE2Bioray LaboratoriesStarted 2022-11-02
BRL-101
Beta-ThalassemiaThalassemiaGenetic Diseases, Inborn

Evaluation of Safety and Efficacy of CTX001 in Pediatric Participants With Transfusion-Dependent β-Thalassemia (TDT)

ACTIVE NOT RECRUITING
NCT05356195Phase PHASE3Vertex Pharmaceuticals IncorporatedStarted 2022-05-03
CTX001
Sickle Cell Disease

Gene Transfer for Sickle Cell Disease

ACTIVE NOT RECRUITING
NCT03282656Phase PHASE1David WilliamsStarted 2018-02-13
single infusion of autologous bone marrow derived CD34+ HSC cells transduced with the lentiviral vector containing a short-hairpin RNA targeting BCL11a
Beta-ThalassemiaThalassemiaHematologic Diseases

Evaluation of Efficacy and Safety of a Single Dose of CTX001 in Participants With Transfusion-Dependent β-Thalassemia and Severe Sickle Cell Disease

RECRUITING
NCT05477563Phase PHASE3Vertex Pharmaceuticals IncorporatedStarted 2022-08-02
CTX001
Sickle Cell Disease

Long-term Follow-up (LTFU) of Patients Treated With Genome-edited Autologous Hematopoietic Stem and Progenitor Cells (HSPC)

ACTIVE NOT RECRUITING
NCT06155500Phase PHASE1Novartis PharmaceuticalsStarted 2024-04-16
OTQ923
Fetal HemoglobinThalassemia MajorGenetic Disease

Unraveling the Impact of Thalidomide at Diverse Doses in Transfusion Dependent Beta Thalassemia

RECRUITING
NCT06490627Phase PHASE2National Institute of Blood and Marrow Transplant (NIBMT), PakistanStarted 2024-04-22
ThalidomideThalidomide
16P11.2 Deletion Syndrome16p11.2 Duplications1Q21.1 Deletion

Online Study of People Who Have Genetic Changes and Features of Autism: Simons Searchlight

RECRUITING
NCT01238250Simons SearchlightStarted 2010-10
Sickle Cell Disease

A Gene Transfer Study Inducing Fetal Hemoglobin in Sickle Cell Disease (GRASP, BMT CTN 2001)

ACTIVE NOT RECRUITING
NCT05353647Phase PHASE2David WilliamsStarted 2022-07-12
Autologous CD34+ HSC cells transduced with the lentiviral vector containing a shRNA targeting BCL11a
Sickle Cell DiseaseSickle Cell Anemia (HbSS, or HbSβ-thalassemia0)Beta-Thalassemia

Hematopoietic Stem Cell BCL11A Enhancer Gene Editing for Severe β-Hemoglobinopathies

RECRUITING
NCT06647979Phase PHASE1Daniel BauerStarted 2025-12-01
autologous bone marrow derived CD34+ HSPCs electroporated with BCL11A enhancer targeting Cas9 ribonucleoproteinSequencing Assay for Variant rs114518452
Sickle Cell DiseaseSickle Cell Anemia (HbSS, or HbSβ-thalassemia0)

Hematopoietic Stem Cell BCL11A Enhancer Gene Editing for Sickle Cell Disease

NOT YET RECRUITING
NCT07708350Phase PHASE1Daniel BauerStarted 2026-08
autologous bone marrow derived CD34+ HSPCs electroporated with BCL11A enhancer targeting Cas9 ribonucleoproteinSequencing Assay for Variant rs114518452
Sickle Cell DiseaseHydroxyurea FailureHydroxyurea Intolerance

Evaluation of Safety and Efficacy of CTX001 in Pediatric Participants With Severe Sickle Cell Disease (SCD)

ACTIVE NOT RECRUITING
NCT05329649Phase PHASE3Vertex Pharmaceuticals IncorporatedStarted 2022-05-02
CTX001