DNMT3B Chromosome 20

DNA methyltransferase 3 beta
85 variants 85 Health Risk

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What This Gene Does
CpG methylation is an epigenetic modification that is important for embryonic development, imprinting, and X-chromosome inactivation. Studies in mice have demonstrated that DNA methylation is required for mammalian development. This gene encodes a DNA methyltransferase which is thought to function in de novo methylation, rather than maintenance methylation. The protein localizes primarily to the nucleus and its expression is developmentally regulated. Mutations in this gene cause the immunodeficiency-centromeric instability-facial anomalies (ICF) syndrome. Eight alternatively spliced transcript variants have been described. The full length sequences of variants 4 and 5 have not been determined. [provided by RefSeq, May 2011]
Gene Info
Gene Group
"PWWP domain containing|7BS C5-cytosine DNA/RNA methyltransferases|DNA/RNA methyltransferases"
Locus Type
gene with protein product
Location
20q11.21
Ensembl
ENSG00000088305
Associated Conditions (10)
Centromeric instability of chromosomes 1
9 and 16 and immunodeficiency
Immunodeficiency-centromeric instability-facial anomalies syndrome 1
Inborn genetic diseases
Kabuki syndrome 1
DNMT3B-related disorder
Facioscapulohumeral muscular dystrophy 4
digenic
See cases
Colon adenocarcinoma
Key Variants
RS111877243
Conflicting classifications of pathogenicity
Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
Health Risk
RS138596278
Conflicting classifications of pathogenicity
Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Centromeric instability of chromosomes 1
Health Risk
RS138805251
Conflicting classifications of pathogenicity
Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
Health Risk
RS139777286
Conflicting classifications of pathogenicity
Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Inborn genetic diseases
Health Risk
RS140518698
Conflicting classifications of pathogenicity
Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
Health Risk
RS140714949
Conflicting classifications of pathogenicity
Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
Health Risk
RS143448185
Conflicting classifications of pathogenicity
Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
Health Risk
RS143462810
Conflicting classifications of pathogenicity
Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
Health Risk
RS143847495
Conflicting classifications of pathogenicity
Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
Health Risk
RS145632647
Conflicting classifications of pathogenicity
Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
Health Risk
RS146299831
Conflicting classifications of pathogenicity
Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
Health Risk
RS147402935
Conflicting classifications of pathogenicity
Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
Health Risk
All Variants (85)
RSID Category Clinical Significance Conditions
RS111877243 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
RS138596278 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Centromeric instability of chromosomes 1
RS138805251 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
RS139777286 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Inborn genetic diseases
RS140518698 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS140714949 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS143448185 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS143462810 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS143847495 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
RS145632647 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
RS146299831 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS147402935 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS147945634 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
RS148646143 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS150148922 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS150718732 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS151128145 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS182618840 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS1979990258 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS1981037102 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Centromeric instability of chromosomes 1
RS200349528 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS200508870 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS200921151 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
RS201067137 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS202113400 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS35846833 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
RS368295290 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS369336236 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Inborn genetic diseases
RS370659978 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
RS373811098 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS374913902 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS375171362 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Inborn genetic diseases
RS377138079 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS538400808 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Inborn genetic diseases
RS544561079 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS556202476 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS563597562 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Inborn genetic diseases
RS564154904 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS61758433 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS746731138 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Facioscapulohumeral muscular dystrophy 4, digenic
RS747182299 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Centromeric instability of chromosomes 1
RS752495145 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
RS756972254 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
RS759448571 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Centromeric instability of chromosomes 1
RS767814649 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Inborn genetic diseases
RS770040598 Health Risk Conflicting classifications of pathogenicity Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Inborn genetic diseases
RS866792483 Health Risk Conflicting classifications of pathogenicity Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Immunodeficiency-centromeric instability-facial anomalies syndrome 1
RS1057518176 Health Risk Likely pathogenic
RS1191203668 Health Risk Likely pathogenic Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency, Centromeric instability of chromosomes 1
RS121908946 Health Risk Likely pathogenic Immunodeficiency-centromeric instability-facial anomalies syndrome 1, Centromeric instability of chromosomes 1, 9 and 16 and immunodeficiency
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