Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations.

Zervas, M

Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations. [electronic resource] - Journal of neuropathology and experimental neurology Jan 2001 - 49-64 p. digital

Publication Type: Comparative Study; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.

0022-3069

10.1093/jnen/60.1.49 doi


Animals
Antigens, CD--metabolism
Axons--pathology
Cats
Cerebral Cortex--metabolism
Child
Child, Preschool
Cholesterol--metabolism
Dendrites--pathology
Esterification
Filipin--metabolism
G(M2) Ganglioside--metabolism
Gangliosides--metabolism
Genotype
Humans
Intracellular Signaling Peptides and Proteins
Lysosomal Membrane Proteins
Membrane Glycoproteins--metabolism
Mice
Mutation
Neurons--metabolism
Niemann-Pick C1 Protein
Niemann-Pick Diseases--genetics
Parvalbumins--metabolism
Proteins--genetics
Reference Values