ABCB6 Chromosome 2

ATP binding cassette subfamily B member 6 (LAN blood group)
23 variants 18 Health Risk 5 Trait

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What This Gene Does
This gene encodes a member of the ATP-binding cassette (ABC) transporter superfamily. ABC proteins transport various molecules across extra- and intra-cellular membranes. This protein is a member of the heavy metal importer subfamily and plays a role in porphyrin transport. This gene is the molecular basis of the Langereis (Lan) blood group antigen and mutations in this gene underlie familial pseudohyperkalemia and dyschromatosis universalis hereditaria. [provided by RefSeq, Mar 2017]
Gene Info
Gene Group
"Blood group antigens|ATP binding cassette subfamily B"
Locus Type
gene with protein product
Location
2q35
Ensembl
ENSG00000115657
Associated Conditions (14)
Protoporphyria
erythropoietic
1
Variegate porphyria
Acute intermittent porphyria
Hereditary coproporphyria
Microphthalmia
isolated
with coloboma 7
ABCB6-related disorder
Inborn genetic diseases
Dyschromatosis universalis hereditaria 3
Langereis blood group
Familial pseudohyperkalemia
Key Variants
All Variants (23)
RSID Category Clinical Significance Conditions
RS13402964 Health Risk Conflicting classifications of pathogenicity
RS145526996 Health Risk Conflicting classifications of pathogenicity Protoporphyria, erythropoietic, 1
RS150221689 Health Risk Conflicting classifications of pathogenicity Microphthalmia, isolated, with coloboma 7
RS201104967 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS201416447 Health Risk Conflicting classifications of pathogenicity
RS377591749 Health Risk Conflicting classifications of pathogenicity ABCB6-related disorder, ABCB6-related disorder
RS541845688 Health Risk Conflicting classifications of pathogenicity Acute intermittent porphyria, Acute intermittent porphyria
RS61733629 Health Risk Conflicting classifications of pathogenicity Dyschromatosis universalis hereditaria 3, Microphthalmia, isolated
RS763768429 Health Risk Conflicting classifications of pathogenicity Dyschromatosis universalis hereditaria 3, Familial pseudohyperkalemia, Microphthalmia
RS770921703 Health Risk Conflicting classifications of pathogenicity ABCB6-related disorder, ABCB6-related disorder
RS201100181 Health Risk Likely pathogenic Dyschromatosis universalis hereditaria 3, Dyschromatosis universalis hereditaria 3
RS764893806 Health Risk Likely pathogenic Familial pseudohyperkalemia, Langereis blood group, Microphthalmia
RS387906910 Health Risk Pathogenic Microphthalmia, isolated, with coloboma 7
RS397514756 Health Risk Pathogenic Dyschromatosis universalis hereditaria 3, Dyschromatosis universalis hereditaria 3
RS397514757 Health Risk Pathogenic Dyschromatosis universalis hereditaria 3, Dyschromatosis universalis hereditaria 3
RS397514758 Health Risk Pathogenic Dyschromatosis universalis hereditaria 3, Dyschromatosis universalis hereditaria 3
RS754667801 Health Risk Pathogenic Familial pseudohyperkalemia, Familial pseudohyperkalemia
RS796065353 Health Risk Pathogenic Dyschromatosis universalis hereditaria 3, Dyschromatosis universalis hereditaria 3
RS1559234527 Trait Affects Langereis blood group, Langereis blood group
RS387906908 Trait Affects Langereis blood group, Langereis blood group
RS387906909 Trait Affects Langereis blood group, Langereis blood group
RS765925019 Trait Affects Langereis blood group, Langereis blood group
RS879255549 Trait Affects Langereis blood group, Langereis blood group
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