ZBTB1

zinc finger and BTB domain containing 1, the group of BTB domain containing|Zinc fingers C2H2-type

Basic information

Region (hg38): 14:64503712-64533690

Links

ENSG00000126804NCBI:22890OMIM:616578HGNC:20259Uniprot:Q9Y2K1AlphaFoldGenCCjaxSfariGnomADPubmedClinVar

Phenotypes

GenCC

Source: genCC

No genCC data.

ClinVar

This is a list of variants' phenotypes submitted to ClinVar and linked to the ZBTB1 gene.

  • not_specified (50 variants)

Variants pathogenicity by type

Statistics on ClinVar variants can assist in determining whether a specific variant type in the ZBTB1 gene is commonly pathogenic or not. These statistics are base on transcript: NM_001123329.2. Only rare variants are included in the table.

In the table, we include only reliable ClinVar variants with their consequences to MANE Select, Mane Plus Clinical transcripts, or transcripts with TSL equals 1. Click the count to view the source variants.

Warning: slight differences between displayed counts and the number of variants in ClinVar may occur, primarily due to (1) the application of a different transcript and/or consequence by our variant effect predictor or (2) differences in clinical significance: we classify Benign/Likely benign variants as Likely benign and Pathogenic/Likely pathogenic variants as Likely pathogenic.

EffectPLPVUSLBBSum
synonymous
0
missense
48
clinvar
2
clinvar
50
nonsense
0
start loss
0
frameshift
0
splice donor/acceptor (+/-2bp)
0
Total 0 0 48 2 0
Loading clinvar variants...

GnomAD

Source: gnomAD

GeneTypeBio TypeTranscript Coding Exons Length
ZBTB1protein_codingprotein_codingENST00000394712 129979
pLI Probability
LOF Intolerant
pRec Probability
LOF Recessive
Individuals with
no LOFs
Individuals with
Homozygous LOFs
Individuals with
Heterozygous LOFs
Defined p
0.9940.00593125735071257420.0000278
Z-Score Observed Expected Observed/Expected Mutation Rate Total Possible in Transcript
Missense2.972113720.5670.00001854833
Missense in Polyphen60152.480.393492039
Synonymous1.99991280.7760.000006201224
Loss of Function4.17224.10.08310.00000151316

LoF frequencies by population

EthnicitySum of pLOFs p
African & African-American0.000.00
Ashkenazi Jewish0.000.00
East Asian0.0001090.000109
Finnish0.000.00
European (Non-Finnish)0.00003520.0000352
Middle Eastern0.0001090.000109
South Asian0.00003270.0000327
Other0.000.00

dbNSFP

Source: dbNSFP

Function
FUNCTION: Acts as a transcriptional repressor (PubMed:20797634). Represses cAMP-responsive element (CRE)-mediated transcriptional activation (PubMed:21706167). In addition, has a role in translesion DNA synthesis. Requires for UV-inducible RAD18 loading, PCNA monoubiquitination, POLH recruitment to replication factories and efficient translesion DNA synthesis (PubMed:24657165). Plays a key role in the transcriptional regulation of T lymphocyte development (By similarity). {ECO:0000250|UniProtKB:Q91VL9, ECO:0000269|PubMed:20797634, ECO:0000269|PubMed:21706167, ECO:0000269|PubMed:24657165}.;

Recessive Scores

pRec
0.104

Intolerance Scores

loftool
0.476
rvis_EVS
-0.25
rvis_percentile_EVS
35.99

Haploinsufficiency Scores

pHI
0.463
hipred
Y
hipred_score
0.732
ghis
0.610

Essentials

essential_gene_CRISPR
N
essential_gene_CRISPR2
S
essential_gene_gene_trap
K
gene_indispensability_pred
E
gene_indispensability_score
0.746

Gene Damage Prediction

AllRecessiveDominant
MendelianMediumMediumMedium
Primary ImmunodeficiencyMediumMediumMedium
CancerMediumMediumMedium

Mouse Genome Informatics

Gene name
Zbtb1
Phenotype
immune system phenotype; hematopoietic system phenotype; endocrine/exocrine gland phenotype;

Gene ontology

Biological process
negative regulation of transcription by RNA polymerase II;positive regulation of T cell mediated immunity;DNA repair;chromatin remodeling;cellular response to DNA damage stimulus;translesion synthesis;B cell differentiation;positive regulation of natural killer cell differentiation;T cell differentiation in thymus;cellular response to UV;mRNA transcription by RNA polymerase II;innate immune response;positive regulation of T cell differentiation;thymus development;protein homooligomerization;positive regulation of pro-T cell differentiation
Cellular component
nucleus;nucleoplasm;nuclear body;nuclear membrane
Molecular function
DNA-binding transcription factor activity, RNA polymerase II-specific;DNA binding;protein binding;protein homodimerization activity;metal ion binding;protein heterodimerization activity;K63-linked polyubiquitin modification-dependent protein binding