Alterations in mitochondrial number and function in Alzheimer's disease fibroblasts.

Abstract:

:Mitochondrial dysfunction is observed in brains of Alzheimer's Disease patients as well as many rodent model systems including those modeling mutations in preseinilin 1 (PSEN1). The aim of our study was to characterize mitochondrial function and number in fibroblasts from AD patients with PSEN1 mutations. We used biochemical assays, metabolic profiling and fluorescent labeling to assess mitochondrial number and function in fibroblasts from three AD patients compared to fibroblasts from three controls. The mutant AD fibroblasts had increased Aβ42 relative to controls along with reduction in ATP, basal and maximal mitochondrial respiration as well as impaired spare mitochondrial respiratory capacity. Fluorescent staining and expression of genes encoding electron transport chain enzymes showed diminished mitochondrial content in the AD fibroblasts. This study demonstrates that mitochondrial dysfunction is observable in AD fibroblasts and provides evidence that this model system could be useful as a tool to screen disease-modifying compounds.

journal_name

Metab Brain Dis

journal_title

Metabolic brain disease

authors

Gray NE,Quinn JF

doi

10.1007/s11011-015-9667-z

subject

Has Abstract

pub_date

2015-10-01 00:00:00

pages

1275-8

issue

5

eissn

0885-7490

issn

1573-7365

journal_volume

30

pub_type

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