Cargando…
Modified Atkins diet induces subacute selective ragged‐red‐fiber lysis in mitochondrial myopathy patients
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 eff...
Autores principales: | , , , , , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2016
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5090657/ https://www.ncbi.nlm.nih.gov/pubmed/27647878 http://dx.doi.org/10.15252/emmm.201606592 |
_version_ | 1782464429816807424 |
---|---|
author | Ahola, Sofia Auranen, Mari Isohanni, Pirjo Niemisalo, Satu Urho, Niina Buzkova, Jana Velagapudi, Vidya Lundbom, Nina Hakkarainen, Antti Muurinen, Tiina Piirilä, Päivi Pietiläinen, Kirsi H Suomalainen, Anu |
author_facet | Ahola, Sofia Auranen, Mari Isohanni, Pirjo Niemisalo, Satu Urho, Niina Buzkova, Jana Velagapudi, Vidya Lundbom, Nina Hakkarainen, Antti Muurinen, Tiina Piirilä, Päivi Pietiläinen, Kirsi H Suomalainen, Anu |
author_sort | Ahola, Sofia |
collection | PubMed |
description | 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 1.5–2 weeks 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. |
format | Online Article Text |
id | pubmed-5090657 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | John Wiley and Sons Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-50906572016-11-25 Modified Atkins diet induces subacute selective ragged‐red‐fiber lysis in mitochondrial myopathy patients Ahola, Sofia Auranen, Mari Isohanni, Pirjo Niemisalo, Satu Urho, Niina Buzkova, Jana Velagapudi, Vidya Lundbom, Nina Hakkarainen, Antti Muurinen, Tiina Piirilä, Päivi Pietiläinen, Kirsi H Suomalainen, Anu EMBO Mol Med Research Articles 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 1.5–2 weeks 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. John Wiley and Sons Inc. 2016-09-19 2016-11 /pmc/articles/PMC5090657/ /pubmed/27647878 http://dx.doi.org/10.15252/emmm.201606592 Text en © 2016 The Authors. Published under the terms of the CC BY 4.0 license This is an open access article under the terms of the Creative Commons Attribution 4.0 (http://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Research Articles Ahola, Sofia Auranen, Mari Isohanni, Pirjo Niemisalo, Satu Urho, Niina Buzkova, Jana Velagapudi, Vidya Lundbom, Nina Hakkarainen, Antti Muurinen, Tiina Piirilä, Päivi Pietiläinen, Kirsi H Suomalainen, Anu Modified Atkins diet induces subacute selective ragged‐red‐fiber lysis in mitochondrial myopathy patients |
title | Modified Atkins diet induces subacute selective ragged‐red‐fiber lysis in mitochondrial myopathy patients |
title_full | Modified Atkins diet induces subacute selective ragged‐red‐fiber lysis in mitochondrial myopathy patients |
title_fullStr | Modified Atkins diet induces subacute selective ragged‐red‐fiber lysis in mitochondrial myopathy patients |
title_full_unstemmed | Modified Atkins diet induces subacute selective ragged‐red‐fiber lysis in mitochondrial myopathy patients |
title_short | Modified Atkins diet induces subacute selective ragged‐red‐fiber lysis in mitochondrial myopathy patients |
title_sort | modified atkins diet induces subacute selective ragged‐red‐fiber lysis in mitochondrial myopathy patients |
topic | Research Articles |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5090657/ https://www.ncbi.nlm.nih.gov/pubmed/27647878 http://dx.doi.org/10.15252/emmm.201606592 |
work_keys_str_mv | AT aholasofia modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT auranenmari modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT isohannipirjo modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT niemisalosatu modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT urhoniina modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT buzkovajana modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT velagapudividya modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT lundbomnina modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT hakkarainenantti modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT muurinentiina modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT piirilapaivi modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT pietilainenkirsih modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients AT suomalainenanu modifiedatkinsdietinducessubacuteselectiveraggedredfiberlysisinmitochondrialmyopathypatients |