Tangier disease

GPTKB entity

Statements (28)
Predicate Object
gptkbp:instanceOf gptkb:genetic_disorder
gptkb:rare_disease
gptkbp:affects lipid metabolism
gptkbp:alsoKnownAs familial alpha-lipoprotein deficiency
gptkbp:characterizedBy gptkb:peripheral_neuropathy
enlarged liver
enlarged spleen
premature atherosclerosis
orange tonsils
very low levels of HDL cholesterol
gptkbp:complication cerebrovascular disease
coronary artery disease
gptkbp:diagnosedBy genetic testing
blood test for HDL cholesterol
gptkbp:firstDescribed 1961
gptkbp:frequency extremely rare
gptkbp:inheritance autosomal recessive
gptkbp:mutationAssociatedWith gptkb:ABCA1_gene
gptkbp:namedAfter Tangier Island, Virginia, USA
gptkbp:OMIM 205400
gptkbp:symptom gptkb:peripheral_neuropathy
hepatosplenomegaly
enlarged yellow-orange tonsils
gptkbp:treatment symptomatic management
dietary modification
gptkbp:bfsParent gptkb:APOA1
gptkbp:bfsLayer 7
https://www.w3.org/2000/01/rdf-schema#label Tangier disease

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