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Altered levels and distribution of amyloid precursor protein and its processing enzymes in Niemann-Pick type C1-deficient mouse brains by A. Kodam; M. Maulik; K. Peake; A. Amritraj; K. S. Vetrivel; G. Thinakaran; J. E. Vance; S. Kar is a Neuroscience article available to read on EtoBox.

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## Abstract Niemann‐Pick type C (NPC) disease is an autosomal recessive neurodegenerative disorder characterized by intracellular accumulation of cholesterol and glycosphingolipids in many tissues including the brain. The disease is caused by mutations of either __NPC1__ or __NPC2__ gene and is accompanied by a severe loss of neurons in the cerebellum, but not in the hippocampus. NPC pathology exhibits some similarities with Alzheimer's disease, including increased levels of amyloid β (Aβ)‐related peptides in vulnerable brain regions, but very little is known about the expression of amyloid precursor protein (APP) or APP secretases in NPC disease. In this article, we evaluated age‐related alterations in the level/distribution of APP and its processing enzymes, β‐ and γ‐secretases, in the hippocampus and cerebellum of __Npc1__^−/−^ mice, a well‐established model of NPC pathology. Our results show that levels and expression of APP and β‐secretase are elevated in the cerebellum prior to changes in the hippocampus, whereas γ‐secretase components are enhanced in both brain regions at the same time in __Npc1__^−/−^ mice. Interestingly, a subset of reactive astrocytes in __Npc1__^−/−^ mou

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Author

A. Kodam; M. Maulik; K. Peake; A. Amritraj; K. S. Vetrivel; G. Thinakaran; J. E. Vance; S. Kar

Publisher

John Wiley and Sons; Wiley (John Wiley & Sons); John Wiley & Sons Inc.; Wiley; Research Square (ISSN 0894-1491)

Published

2010

Language

EN

Field

Neuroscience (Life Sciences)