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      The “Down's” Side of Mitochondria

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      Developmental Cell
      Elsevier BV

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          Abstract

          The mechanisms involved in the development of Alzheimer disease-type pathology in patients with Down's syndrome are unclear. However, a paper published in the February 28 issue of Neuron indicates that mitochondrial dysfunction is pivotally involved.

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          Altered metabolism of the amyloid beta precursor protein is associated with mitochondrial dysfunction in Down's syndrome.

          Most Down's syndrome (DS) patients develop Alzheimer's disease (AD) neuropathology. Astrocyte and neuronal cultures derived from fetal DS brain show alterations in the processing of amyloid beta precursor protein (AbetaPP), including increased levels of AbetaPP and C99, reduced levels of secreted AbetaPP (AbetaPPs) and C83, and intracellular accumulation of insoluble Abeta42. This pattern of AbetaPP processing is recapitulated in normal astrocytes by inhibition of mitochondrial metabolism, consistent with impaired mitochondrial function in DS astrocytes. Intracellular Abeta42 and reduced AbetaPPs are also detected in DS and AD brains. The survival of DS neurons is markedly increased by recombinant or astrocyte-produced AbetaPPs, suggesting that AbetaPPs may be a neuronal survival factor. Thus, mitochondrial dysfunction in DS may lead to intracellular deposition of Abeta42, reduced levels of AbetaPPs, and a chronic state of increased neuronal vulnerability.
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            Decreased levels of complex III core protein 1 and complex V beta chain in brains from patients with Alzheimer's disease and Down syndrome.

            Ubiquinol:cytochrome c oxidoreductase (complex III) and ATP synthase (complex V) are important enzymes in the mitochondrial electron transport chain. Defects in mitochondrial respiratory enzymes have been reported for several neurodegenerative diseases. In this study, we applied the proteomic approach to investigate protein levels of complex III core protein and complex V beta chain in brain regions of Alzheimer's disease (AD) and Down syndrome (DS) patients. Complex III core protein 1 was significantly reduced in the temporal cortex of AD patients. Complex V beta chain was significantly reduced in the frontal cortex of DS patients. We conclude that decreased mitochondrial respiratory enzymes could contribute to the impairment of energy metabolism observed in DS. These decreases could also cause the generation of reactive oxygen species and neuronal cell death (apoptosis) in DS as well as AD.
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              Amyloid-β junkies

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                Author and article information

                Journal
                Developmental Cell
                Developmental Cell
                Elsevier BV
                15345807
                March 2002
                March 2002
                : 2
                : 3
                : 255-256
                Article
                10.1016/S1534-5807(02)00139-9
                11879629
                6301ff3d-8fb8-44e0-99ca-b667f672a228
                © 2002

                http://www.elsevier.com/tdm/userlicense/1.0/

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