Niemann-Pick Disease, Type C
Synonyms
Neurovisceral Storage Disease with Vertical Supranuclear Ophthalmoplegia
Niemann Pick Disease Type D
Niemann Pick Disease with Cholesterol Esterification Block
Niemann Pick Disease without Sphingomyelinase Deficiency
Niemann Pick Disease, Chronic Neuronopathic Form
Niemann Pick Disease, Nova Scotian
Niemann Pick Disease, Type C
Niemann Pick Disease, Type D
Niemann Pick disease, Subacute Juvenile Form
Niemann Pick's Disease Type C
Niemann Pick's Disease Type D
Niemann-Pick Disease Type D
Niemann-Pick Disease with Cholesterol Esterification Block
Niemann-Pick Disease without Sphingomyelinase Deficiency
Niemann-Pick Disease, Chronic Neuronopathic Form
Niemann-Pick Disease, Nova Scotian
Niemann-Pick Disease, Type C1
Niemann-Pick Disease, Type D
Niemann-Pick disease, Subacute Juvenile Form
Niemann-Pick's Disease Type C
Niemann-Pick's Disease Type D
Nova Scotia (Type D) Form of Niemann-Pick Disease
Nova Scotia Niemann Pick Disease (Type D)
Nova Scotia Niemann-Pick Disease (Type D)
An autosomal recessive lipid storage disorder that is characterized by accumulation of CHOLESTEROL and SPHINGOMYELINS in cells of the VISCERA and the CENTRAL NERVOUS SYSTEM. Type C (or C1) and type D are allelic disorders caused by mutation of gene (NPC1) encoding a protein that mediate intracellular cholesterol transport from lysosomes. Clinical signs include hepatosplenomegaly and chronic neurological symptoms. Type D is a variant in people with a Nova Scotia ancestry.