Defining the Role of the Bcl-2 Family Proteins in Huntington's Disease

Overview

Journal Cell Death Dis

Specialties Cell Biology
General Medicine

Date 2013 Aug 17

PMID 23949221

Citations 20

Authors

J Sassone

A Maraschi

F Sassone

V Silani

A Ciammola

Affiliations

Soon will be listed here.

Abstract

B-cell lymphoma 2 (Bcl-2) family proteins regulate survival, mitochondria morphology dynamics and metabolism in many cell types including neurons. Huntington's disease (HD) is a neurodegenerative disorder caused by an expanded CAG repeat tract in the IT15 gene that encodes for the protein huntingtin (htt). In vitro and in vivo models of HD and HD patients' tissues show abnormal mitochondrial function and increased cell death rates associated with alterations in Bcl-2 family protein expression and localization. This review aims to draw together the information related to Bcl-2 family protein alterations in HD to decipher their potential role in mutated htt-related cell death and mitochondrial dysfunction.

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