TY - JOUR
T1 - Dissembled DJ-1 high molecular weight complex in cortex mitochondria from Parkinson's disease patients
AU - Nural, Hikmet
AU - He, Ping
AU - Beach, Thomas
AU - Sue, Lucia
AU - Xia, Weiming
AU - Shen, Yong
N1 - Funding Information:
This work is supported in part by the grant from Arizona Biomedical Research Commission Arizona Parkinson Consortium AZPD0011 (TB, YS), NIHAG025888 (YS) and Parkinson's disease Foundation (YS) and Harvard Center for Neurodegeneration and Repair (WX). We thank Mr. Zhenyu Zhong for providing technical assistance in this study and Ms. Gina Ciavarella and Ms. Nezahet Mutlu for their editorial assistance.
PY - 2009
Y1 - 2009
N2 - The PARK7 gene encodes a protein, DJ-1, with several functions such as protection of cells from oxidative stress, sperm maturation and fertilization, and chaperone activity. Mutations in the PARK7 gene are associated with autosomal recessive early-onset Parkinson's disease (PD). DJ-1 has been reported to be expressed in multiple cells in the central nerve system. Here, by using both native and denatured Western blots, we examined levels of total DJ-1 and high molecular weight complexes of DJ-1 (HMW) in both the substantia nigra and cortex from rapidly autopsied 18 PD and 9 non-pathological control (NPC) brains. We have discovered that the level of total DJ-1 protein is significantly reduced in the substantia nigra in brains of sporadic PD patients. Moreover, in the PD cortex mitochondria fraction, the HMW DJ-1 complex is significantly lower than in the NPC. These results suggest abnormal DJ-1 expression levels and DJ-1 complex changes may contribute to PD pathogenesis.
AB - The PARK7 gene encodes a protein, DJ-1, with several functions such as protection of cells from oxidative stress, sperm maturation and fertilization, and chaperone activity. Mutations in the PARK7 gene are associated with autosomal recessive early-onset Parkinson's disease (PD). DJ-1 has been reported to be expressed in multiple cells in the central nerve system. Here, by using both native and denatured Western blots, we examined levels of total DJ-1 and high molecular weight complexes of DJ-1 (HMW) in both the substantia nigra and cortex from rapidly autopsied 18 PD and 9 non-pathological control (NPC) brains. We have discovered that the level of total DJ-1 protein is significantly reduced in the substantia nigra in brains of sporadic PD patients. Moreover, in the PD cortex mitochondria fraction, the HMW DJ-1 complex is significantly lower than in the NPC. These results suggest abnormal DJ-1 expression levels and DJ-1 complex changes may contribute to PD pathogenesis.
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U2 - 10.1186/1750-1326-4-23
DO - 10.1186/1750-1326-4-23
M3 - Article
C2 - 19497122
AN - SCOPUS:68149083132
SN - 1750-1326
VL - 4
JO - Molecular Neurodegeneration
JF - Molecular Neurodegeneration
IS - 1
M1 - 23
ER -