Modified Atkins diet induces subacute selective ragged-red-fiber lysis in mitochondrial myopathypatients

Show full item record



Permalink

http://hdl.handle.net/10138/175909

Citation

Ahola , S , Auranen , M , Isohanni , P , Niemisalo , S , Urho , N , Buzkova , J , Velagapudi , V , Lundbom , N , Hakkarainen , A , Muurinen , T , Piirilä , P , Pietilainen , K H & Suomalainen , A 2016 , ' Modified Atkins diet induces subacute selective ragged-red-fiber lysis in mitochondrial myopathypatients ' , EMBO molecular medicine , vol. 8 , no. 11 , pp. 1234-1247 . https://doi.org/10.15252/emmm.201606592

Title: Modified Atkins diet induces subacute selective ragged-red-fiber lysis in mitochondrial myopathypatients
Author: Ahola, Sofia; Auranen, Mari; Isohanni, Pirjo; Niemisalo, Satu; Urho, Niina; Buzkova, Jana; Velagapudi, Vidya; Lundbom, Nina; Hakkarainen, Antti; Muurinen, Tiina; Piirilä, Päivi; Pietilainen, Kirsi H.; Suomalainen, Anu
Contributor: University of Helsinki, Research Programs Unit
University of Helsinki, Research Programs Unit
University of Helsinki, Research Programme for Molecular Neurology
University of Helsinki, Research Programs Unit
University of Helsinki, Institute for Molecular Medicine Finland
University of Helsinki, Clinicum
University of Helsinki, Clinicum
University of Helsinki, Department of Diagnostics and Therapeutics
University of Helsinki, Clinicum
University of Helsinki, Clinicum
University of Helsinki, Clinicum
Date: 2016-11
Language: eng
Number of pages: 14
Belongs to series: EMBO molecular medicine
ISSN: 1757-4676
URI: http://hdl.handle.net/10138/175909
Abstract: Mitochondrial myopathy (MM) with progressive external ophthalmoplegia (PEO) is a common manifestation of mitochondrial disease in adulthood, for which there is no curative therapy. In mice with MM, ketogenic diet significantly delayed progression of the disease. We asked in this pilot study what effects high-fat, low-carbohydrate modified Atkins diet (mAD) had for PEO/MM patients and control subjects and followed up the effects by clinical, morphological, transcriptomic, and metabolomic analyses. All of our five patients, irrespective of genotype, showed a subacute response after 1.5-2weeks of diet, with progressive muscle pain and leakage of muscle enzymes, leading to premature discontinuation of the diet. Analysis of muscle ultrastructure revealed selective fiber damage, especially in the ragged-red-fibers (RRFs), a MM hallmark. Two years of follow-up showed improvement of muscle strength, suggesting activation of muscle regeneration. Our results indicate that (i) nutrition can modify mitochondrial disease progression, (ii) dietary counseling should be part of MM care, (iii) short mAD is a tool to induce targeted RRF lysis, and (iv) mAD, a common weight-loss method, may induce muscle damage in a population subgroup.
Subject: mitochondrial myopathy
modified Atkins diet
PEO
ragged-red-fibers
LOW-CARBOHYDRATE-DIET
INSULIN-RESISTANCE
KETOGENIC DIET
OBESE-PATIENTS
DNA DELETIONS
QUANTIFICATION
PREVALENCE
DISORDERS
CHILDREN
DISEASES
3112 Neurosciences
3111 Biomedicine
416 Food Science
Rights:


Files in this item

Total number of downloads: Loading...

Files Size Format View
emmm201606592.pdf 5.548Mb PDF View/Open

This item appears in the following Collection(s)

Show full item record