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Mitochondrial Functions and Oxidative Stress in ALS Patients

2014-08-27 03:44:21 | BioPortfolio

Summary

In Amyotrophic Lateral Sclerosis (ALS), malnutrition is frequent (16 to 50 % of the patients) and is an independent prognostic factor. One of the implicated factors is the increase of resting energy expenditure (REE) which can be found in about 50 % of ALS patients. The origin of this hypermetabolism is currently unknown but could be located in the mitochondria. In fact, some studies have found mitochondrial abnormalities and the existence of an oxidative stress. Thus, the aim of this study is to characterize the mitochondrial abnormalities and the oxidant/antioxidant status of ALS patients and to determine their relationship with the metabolic status, hypermetabolism or normometabolism. Three groups of patients will be studied : 20 hypermetabolic ALS patients, 20 normometabolic ALS patients and 20 healthy volunteers paired for age and sex.

Description

In Amyotrophic Lateral Sclerosis (ALS), malnutrition is frequent (16 to 50 % of the patients) and is an independent prognostic factor. One of the implicated factors is the increase of resting energy expenditure (REE) which can be found in about 50 % of ALS patients. The origin of this hypermetabolism is currently unknown but could be located in the mitochondria. In fact, some studies have found mitochondrial abnormalities and the existence of an oxidative stress. Thus, the aim of this study is to characterize the mitochondrial abnormalities and the oxidant/antioxidant status of ALS patients and to determine their relationship with the metabolic status, hypermetabolism or normometabolism. Three groups of patients will be studied : 20 hypermetabolic ALS patients, 20 normometabolic ALS patients and 20 healthy volunteers paired for age and sex.

Study Design

Time Perspective: Prospective

Conditions

Amyotrophic Lateral Sclerosis

Intervention

Mitochondrial functions and oxidative stress

Location

Clermont-Ferrand University Hospital
Clermont-Ferrand
Auvergne
France
63000

Status

Recruiting

Source

University Hospital, Clermont-Ferrand

Results (where available)

View Results

Links

Published on BioPortfolio: 2014-08-27T03:44:21-0400

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Medical and Biotech [MESH] Definitions

A highly-conserved AAA ATPase that functions in the biogenesis of the transitional ENDOPLASMIC RETICULUM and fragmentation and reassembly of the GOLGI APPARATUS during MITOSIS. It also functions in a complex with UFD1L and NPLOC4 proteins to export misfolded ubiquitinated proteins from the endoplasmic reticulum and outer mitochondrial membrane to the cytoplasm for degradation by the PROTEASOME and also plays a role in AUTOPHAGY of ubiquitinated proteins. It occurs in neuronal INCLUSION BODIES from patients with AMYOTROPHIC LATERAL SCLEROSIS and LEWY BODIES from PARKINSON DISEASE patients.

A disturbance in the prooxidant-antioxidant balance in favor of the former, leading to potential damage. Indicators of oxidative stress include damaged DNA bases, protein oxidation products, and lipid peroxidation products (Sies, Oxidative Stress, 1991, pxv-xvi).

A glutamate antagonist (RECEPTORS, GLUTAMATE) used as an anticonvulsant (ANTICONVULSANTS) and to prolong the survival of patients with AMYOTROPHIC LATERAL SCLEROSIS.

A superoxide dismutase (SOD1) that requires copper and zinc ions for its activity to destroy SUPEROXIDE FREE RADICALS within the CYTOPLASM. Mutations in the SOD1 gene are associated with AMYOTROPHIC LATERAL SCLEROSIS-1.

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