Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis.

Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis. Research Abstract Details 

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Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis. Abstract Text:

John J Shacka,Kevin A Roth,

Animal models of cathepsin D (CD) deficiency are characterized by a progressive and relentless neurodegenerative phenotype similar to that observed in Neuronal Ceroid Lipofuscinoses (NCL), a group of pediatric neurodegenerative diseases known collectively as Batten Disease. We have shown recently that the targeted deletion of the pro-apoptotic molecule Bax prevents apoptotic markers but not neuron death and neurodegeneration induced by CD deficiency, which suggests that alterations in the macroautophagy-lysosomal degradation pathway can mediate neuron death in NCL/Batten Disease in the absence of apoptosis. Herein, we review CD deficiency in the broader context of NCL and offer potential mechanisms for neuron death and neurodegeneration induced by CD deficiency.

Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis. Publishing Authors By Initials

JJ Shacka,KA Roth,

For similar nervous system: neurons research abstracts see: nervous system: neurons research

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Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis. Journal Published:

PUBLICATION TYPE: Review

Journal: Autophagy

VOLUME: 3

Page Numbers: 474-6

Journal Abbreviation: Autophagy

ISSN: 1554-8627

DAY: 25

MONTH: 04

YEAR: 2007

Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis. Information

Number of References: 24

LANGUAGE: eng

NlmUniqueID: 101265188

Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis. Keywords Mesh Terms:

KEYWORDS: Neurons

MESH TERMS: pathology

Chemical & Substance for Abstract: Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis. Information

Substance Name: Cathepsin D

Registry Number: EC 3.4.23.5

Grant and Affiliation Information for Cathepsin D deficiency and NCL/Batten disease: there's more to death than apoptosis.

AFFILIATION: Department of Pathology, Neuropathology Division, University of Alabama at Birmingham, Birmingham, Alabama 35294-0017, USA. shacka@uab.edu

Country: United States

AGENCY: United States NINDS

GRANT: NS57098

ACRONYM: NS

MEDLINETA: Autophagy

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