TY  - JOUR
AU  - Exner, Nicole
AU  - Müller-Rischart, Anne Kathrin
AU  - Haass, Christian
AU  - Winklhofer, Konstanze F
TI  - Mitochondrial dysfunction in Parkinson's disease: molecular mechanisms and pathophysiological consequences.
JO  - The EMBO journal
VL  - 31
IS  - 14
SN  - 0261-4189
CY  - Hoboken, NJ [u.a.]
PB  - Wiley
M1  - DZNE-2020-02902
SP  - 3038-3062
PY  - 2012
AB  - Neurons are critically dependent on mitochondrial integrity based on specific morphological, biochemical, and physiological features. They are characterized by high rates of metabolic activity and need to respond promptly to activity-dependent fluctuations in bioenergetic demand. The dimensions and polarity of neurons require efficient transport of mitochondria to hot spots of energy consumption, such as presynaptic and postsynaptic sites. Moreover, the postmitotic state of neurons in combination with their exposure to intrinsic and extrinsic neuronal stress factors call for a high fidelity of mitochondrial quality control systems. Consequently, it is not surprising that mitochondrial alterations can promote neuronal dysfunction and degeneration. In particular, mitochondrial dysfunction has long been implicated in the etiopathogenesis of Parkinson's disease (PD), based on the observation that mitochondrial toxins can cause parkinsonism in humans and animal models. Substantial progress towards understanding the role of mitochondria in the disease process has been made by the identification and characterization of genes causing familial variants of PD. Studies on the function and dysfunction of these genes revealed that various aspects of mitochondrial biology appear to be affected in PD, comprising mitochondrial biogenesis, bioenergetics, dynamics, transport, and quality control.
KW  - Animals
KW  - Humans
KW  - Mitochondria: genetics
KW  - Mitochondria: metabolism
KW  - Mitochondria: pathology
KW  - Neurons: metabolism
KW  - Neurons: pathology
KW  - Parkinson Disease: genetics
KW  - Parkinson Disease: metabolism
KW  - Parkinson Disease: pathology
LB  - PUB:(DE-HGF)16
C6  - pmid:22735187
C2  - pmc:PMC3400019
DO  - DOI:10.1038/emboj.2012.170
UR  - https://pub.dzne.de/record/136580
ER  -