Increased activity and altered subcellular distribution of lysosomal enzymes determine neuronal vulnerability in Niemann-Pick type C1-deficient mice.

Amritraj, Asha

Increased activity and altered subcellular distribution of lysosomal enzymes determine neuronal vulnerability in Niemann-Pick type C1-deficient mice. [electronic resource] - The American journal of pathology Dec 2009 - 2540-56 p. digital

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

1525-2191

10.2353/ajpath.2009.081096 doi


Animals
Cathepsin B--metabolism
Cathepsin D--metabolism
Cerebellum--metabolism
Cholesterol--metabolism
Fluorescent Antibody Technique
Hippocampus--metabolism
Immunoblotting
Intracellular Signaling Peptides and Proteins
Lysosomes--enzymology
Mice
Mice, Inbred BALB C
Mice, Knockout
Microscopy, Electron, Transmission
Nerve Degeneration--enzymology
Neurons--enzymology
Niemann-Pick C1 Protein
Niemann-Pick Disease, Type C--enzymology
Proteins--genetics