BBADIS-63907; No. of page: 1; 4C: Biochimica et Biophysica Acta xxx (2014) xxx
Contents lists available at ScienceDirect
Biochimica et Biophysica Acta journal homepage: www.elsevier.com/locate/bbadis
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Preface
Misfolded proteins, mitochondrial dysfunction, and neurodegenerative diseases
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Dr. P. Hemachandra Reddy is an associate scientist in Neuroscience Division of Oregon National Primate Research Center of Oregon Health and Science University. He did his B.Sc. and M.Sc. in Biology from Sri Venkateswara University and M.Phil. in Cytogenetics from Delhi University. He was a commonwealth scholar (1990–1993), earned his Ph.D. in Human Genetics (1994) from University College London, London University. He did his postdoctoral training (1995– 2000) with Dr. Danilo A. Tagle at the National Human Genome Research Institute, NIH. He has worked over 18 years in the areas of aging, mitochondria and neurodegenerative diseases. He has authored and co-authored over 85 peer-reviewed research papers, including highly cited and influential articles in Nature Genetics, Human Molecular Genetics, Trends in Neurosciences, and Trends in Molecular Medicine. Currently, he is an associate/academic/handling editor for 8 journals, and an editorial board for over 20 journals.
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clinical and basic science researchers, and other researchers who are interested in oxidative stress and mitochondrial dysfunction in aging, neurodegenerative diseases, and inherited mitochondrial diseases. I sincerely thank all the contributors for their outstanding articles and our reviewers who provided cogent, timely reviews. I also thank Andy Deelen at Elsevier, Amsterdam; Drs. Jeffrey Keller and Ronald Oude Elferink, Executive Editors; Don Prince, Managing Editor; and all of the production and printing staff for their professional support and help in assembling this special issue.
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Mitochondria are cytoplasmic organelles that are essential for life and death of cells. They perform several cellular functions, including the regulation of intracellular calcium, ATP production, free radical production and scavenging, and the release of proteins that activate the caspase family of proteases. Increasing evidence suggests that oxidative stress and mitochondrial dysfunction are involved in aging, neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, Huntington's disease, multiple sclerosis, cancer, diabetes and obesity, cardiovascular diseases, ischemia, stroke, and alcohol injury. Recent research has found that, in neurodegenerative diseases, mitochondrial dynamics (fission and fusion states) in neurons are imbalanced in a diseased state. The association of mutant proteins with mitochondria has been reported to cause abnormal mitochondrial dynamics, leading to mitochondrial dysfunction and neuronal damage. Recently, progress has been made in better understanding the role of axonal transport of mitochondria and abnormal mitochondrial dynamics in mitochondrial dysfunction. In the last decade, tremendous progress has also been made in terms of developing molecules and antioxidants that target mitochondria and reduce mitochondrial toxicity, that boost and/or maintain the dynamics of mitochondria (fission and fusion balance), and that improve mitochondrial function and neuronal activity in normal and diseased states. The purpose of this special issue is to assess the recent developments in mitochondrial research in order to improve our basic understanding of: 1) mitochondrial structure and function in normal and diseased states, 2) the status of mutant protein(s) associated with mitochondria, abnormal mitochondrial dynamics, and mitochondrial dysfunction, and 3) the development of mitochondria-targeted therapeutics in aging and neurodegenerative diseases. I strongly believe that this special issue, which focuses on misfolded proteins and mitochondrial dysfunction in neurodegenerative diseases, provides timely resources for students,
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Dr. P. Hemachandra Reddy Q1 Available online xxxx
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http://dx.doi.org/10.1016/j.bbadis.2014.03.012 0925-4439/© 2014 Elsevier B.V. All rights reserved.
Please cite this article as: P.H. Reddy, Misfolded proteins, mitochondrial dysfunction, and neurodegenerative diseases, Biochim. Biophys. Acta (2014), http://dx.doi.org/10.1016/j.bbadis.2014.03.012
Contents lists available at ScienceDirect
Biochimica et Biophysica Acta journal homepage: www.elsevier.com/locate/bbadis
2 3
Preface
Misfolded proteins, mitochondrial dysfunction, and neurodegenerative diseases
F
1
20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36
R O
P
37 38 39 40 41 42 43 44 45
D
46
Dr. P. Hemachandra Reddy is an associate scientist in Neuroscience Division of Oregon National Primate Research Center of Oregon Health and Science University. He did his B.Sc. and M.Sc. in Biology from Sri Venkateswara University and M.Phil. in Cytogenetics from Delhi University. He was a commonwealth scholar (1990–1993), earned his Ph.D. in Human Genetics (1994) from University College London, London University. He did his postdoctoral training (1995– 2000) with Dr. Danilo A. Tagle at the National Human Genome Research Institute, NIH. He has worked over 18 years in the areas of aging, mitochondria and neurodegenerative diseases. He has authored and co-authored over 85 peer-reviewed research papers, including highly cited and influential articles in Nature Genetics, Human Molecular Genetics, Trends in Neurosciences, and Trends in Molecular Medicine. Currently, he is an associate/academic/handling editor for 8 journals, and an editorial board for over 20 journals.
E
18 19
T
16 17
C
14 15
E
12 13
R
10 11
clinical and basic science researchers, and other researchers who are interested in oxidative stress and mitochondrial dysfunction in aging, neurodegenerative diseases, and inherited mitochondrial diseases. I sincerely thank all the contributors for their outstanding articles and our reviewers who provided cogent, timely reviews. I also thank Andy Deelen at Elsevier, Amsterdam; Drs. Jeffrey Keller and Ronald Oude Elferink, Executive Editors; Don Prince, Managing Editor; and all of the production and printing staff for their professional support and help in assembling this special issue.
R
8 9
Mitochondria are cytoplasmic organelles that are essential for life and death of cells. They perform several cellular functions, including the regulation of intracellular calcium, ATP production, free radical production and scavenging, and the release of proteins that activate the caspase family of proteases. Increasing evidence suggests that oxidative stress and mitochondrial dysfunction are involved in aging, neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, Huntington's disease, multiple sclerosis, cancer, diabetes and obesity, cardiovascular diseases, ischemia, stroke, and alcohol injury. Recent research has found that, in neurodegenerative diseases, mitochondrial dynamics (fission and fusion states) in neurons are imbalanced in a diseased state. The association of mutant proteins with mitochondria has been reported to cause abnormal mitochondrial dynamics, leading to mitochondrial dysfunction and neuronal damage. Recently, progress has been made in better understanding the role of axonal transport of mitochondria and abnormal mitochondrial dynamics in mitochondrial dysfunction. In the last decade, tremendous progress has also been made in terms of developing molecules and antioxidants that target mitochondria and reduce mitochondrial toxicity, that boost and/or maintain the dynamics of mitochondria (fission and fusion balance), and that improve mitochondrial function and neuronal activity in normal and diseased states. The purpose of this special issue is to assess the recent developments in mitochondrial research in order to improve our basic understanding of: 1) mitochondrial structure and function in normal and diseased states, 2) the status of mutant protein(s) associated with mitochondria, abnormal mitochondrial dynamics, and mitochondrial dysfunction, and 3) the development of mitochondria-targeted therapeutics in aging and neurodegenerative diseases. I strongly believe that this special issue, which focuses on misfolded proteins and mitochondrial dysfunction in neurodegenerative diseases, provides timely resources for students,
N C O
6 7
47 52 48 53 54 49 55 50 56 57 51 58 59 60 61 62 63 64 65 66 67 68 69 70 71
Dr. P. Hemachandra Reddy Q1 Available online xxxx
U
5
O
4
http://dx.doi.org/10.1016/j.bbadis.2014.03.012 0925-4439/© 2014 Elsevier B.V. All rights reserved.
Please cite this article as: P.H. Reddy, Misfolded proteins, mitochondrial dysfunction, and neurodegenerative diseases, Biochim. Biophys. Acta (2014), http://dx.doi.org/10.1016/j.bbadis.2014.03.012
