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Volume 7, Issue 1 (Suppl) J Clin Exp Pathol
ISSN: 2161-0681 JCEP, an open access journal
Pediatric Pathology & Laboratory Medicine 2017
March 15-16, 2017
Page 27
conferenceseries.com
March 15-16, 2017 London, UK
12th International Conference on
Pediatric Pathology & Laboratory Medicine
Electron Paramagnetic Resonance (EPR) spectroscopy for diagnosis and characterization of
mitochondrial dysfunction and diseases
Mitochondrial disease (MD) presents with a wide range of clinical, pathological and biochemical outcomes and is
consequently very difficult to diagnose conclusively. EPR is a magnetic resonance technique that detects and characterizes
unpaired electrons that are present in transition metal ions in certain oxidation states {e.g. Fe(III) , Cu(II) and Mn(II)}, clusters
(e.g., [2Fe2S]+
red, [3Fe4S]+
ox, [4Fe4S] +
red) and free radicals (e.g., UQ•− , FADH•). The mitochondrial respiratory chain complexes
I-IV contain 23 potentially paramagnetic centers that exhibit distinct EPR signals depending on their redox potentials, the
availability of electrons, the catalytic competence of each of the enzymatic complexes and the integrity of the electron transport
chain (ETC). In addition, EPR signals may be observed fromUQ•−, and from the [3Fe4S]+ cluster of m-aconitase that arises due
to oxidative stress. Key factors thought to be involved in the symptoms and pathology of MD is lowered ATP production and the
production of toxic reactive oxygen species (ROS). Either or both of these can occur when electron transfer is impeded due to
lowered expression, lowered activity, or structural alteration of ETC complexes, or compromised ingress or egress of reducing
equivalents. EPR of rapidly-frozen fresh biopsy tissue is uniquely able to provide a snapshot of the electron distribution among
the redox centers in the functioning mitochondrial ETC against a background of other biochemical and pathological assays.
We recently described the first application of this methodology to a rat model of MD and will here describe progress toward
translation of the approach for diagnosis and differentiation of MDs in children.
Biography
Brian Bennett has completed his BA and MA in Natural Sciences from the University of Cambridge and DPhil in Biochemistry from the University of Sussex. Currently, he
is a Chair and Distinguished Professor of Physics at Marquette University, USA.
Brian Bennett
Marquette University, USA
Brian Bennett, J Clin Exp Pathol 2017, 7:1 (Suppl)