Inclusion body myositis – pathomechanism and lessons from genetics

Inclusion body myositis is a rare, late-onset myopathy. Both inflammatory and myodegenerative features play an important role in their pathogenesis. Overlapping clinicopathological entities are the familial inclusion body myopathies with or without dementia. These myopathies share several clinical a...

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Main Authors: Murnyák Balázs, Bodoki Levente, Vincze Melinda, Griger Zoltán, Csonka Tamás, Szepesi Rita, Kurucz Andrea, Dankó Katalin, Hortobágyi Tibor
Format: Article
Language:English
Published: De Gruyter 2015-02-01
Series:Open Medicine
Subjects:
GNE
DES
VCP
Online Access:http://www.degruyter.com/view/j/med.2015.10.issue-1/med-2015-0030/med-2015-0030.xml?format=INT
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spelling doaj-d1ae49101b9542e5ba63cdf5445fa99c2021-10-02T03:10:13ZengDe GruyterOpen Medicine2391-54632015-02-0110110.1515/med-2015-0030med-2015-0030Inclusion body myositis – pathomechanism and lessons from geneticsMurnyák Balázs0Bodoki Levente1Vincze Melinda2Griger Zoltán3Csonka Tamás4Szepesi Rita5Kurucz Andrea6Dankó Katalin7Hortobágyi Tibor8Division of Neuropathology, Institute of PathologyInstitute of Internal Medicine, Third Department of Internal Medicine, Division of Clinical ImmunologyInstitute of Internal Medicine, Third Department of Internal Medicine, Division of Clinical ImmunologyInstitute of Internal Medicine, Third Department of Internal Medicine, Division of Clinical ImmunologyDivision of Neuropathology, Institute of PathologyDepartment of Neurology, University of Debrecen, Faculty of Medicine, Debrecen, HungaryDivision of Neuropathology, Institute of PathologyInstitute of Internal Medicine, Third Department of Internal Medicine, Division of Clinical ImmunologyUniversity of Debrecen, Faculty of Medicine, Institute of Pathology, Division of Neuropathology, 4032 Debrecen, Nagyerdei krt. 98. Tel.: + 36 52 255-248; e-mail: tibor.hortobagyi@kcl.ac.ukInclusion body myositis is a rare, late-onset myopathy. Both inflammatory and myodegenerative features play an important role in their pathogenesis. Overlapping clinicopathological entities are the familial inclusion body myopathies with or without dementia. These myopathies share several clinical and pathological features with the sporadic inflammatory disease. Therefore, better understanding of the genetic basis and pathomechanism of these rare familial cases may advance our knowledge and enable more effective treatment options in sporadic IBM, which is currently considered a relentlessly progressive incurable disease.http://www.degruyter.com/view/j/med.2015.10.issue-1/med-2015-0030/med-2015-0030.xml?format=INTsporadic inclusion body myositis genetics GNE DES VCPIBMPFD TDP-43
collection DOAJ
language English
format Article
sources DOAJ
author Murnyák Balázs
Bodoki Levente
Vincze Melinda
Griger Zoltán
Csonka Tamás
Szepesi Rita
Kurucz Andrea
Dankó Katalin
Hortobágyi Tibor
spellingShingle Murnyák Balázs
Bodoki Levente
Vincze Melinda
Griger Zoltán
Csonka Tamás
Szepesi Rita
Kurucz Andrea
Dankó Katalin
Hortobágyi Tibor
Inclusion body myositis – pathomechanism and lessons from genetics
Open Medicine
sporadic inclusion body myositis
genetics
GNE
DES
VCP
IBMPFD
TDP-43
author_facet Murnyák Balázs
Bodoki Levente
Vincze Melinda
Griger Zoltán
Csonka Tamás
Szepesi Rita
Kurucz Andrea
Dankó Katalin
Hortobágyi Tibor
author_sort Murnyák Balázs
title Inclusion body myositis – pathomechanism and lessons from genetics
title_short Inclusion body myositis – pathomechanism and lessons from genetics
title_full Inclusion body myositis – pathomechanism and lessons from genetics
title_fullStr Inclusion body myositis – pathomechanism and lessons from genetics
title_full_unstemmed Inclusion body myositis – pathomechanism and lessons from genetics
title_sort inclusion body myositis – pathomechanism and lessons from genetics
publisher De Gruyter
series Open Medicine
issn 2391-5463
publishDate 2015-02-01
description Inclusion body myositis is a rare, late-onset myopathy. Both inflammatory and myodegenerative features play an important role in their pathogenesis. Overlapping clinicopathological entities are the familial inclusion body myopathies with or without dementia. These myopathies share several clinical and pathological features with the sporadic inflammatory disease. Therefore, better understanding of the genetic basis and pathomechanism of these rare familial cases may advance our knowledge and enable more effective treatment options in sporadic IBM, which is currently considered a relentlessly progressive incurable disease.
topic sporadic inclusion body myositis
genetics
GNE
DES
VCP
IBMPFD
TDP-43
url http://www.degruyter.com/view/j/med.2015.10.issue-1/med-2015-0030/med-2015-0030.xml?format=INT
work_keys_str_mv AT murnyakbalazs inclusionbodymyositispathomechanismandlessonsfromgenetics
AT bodokilevente inclusionbodymyositispathomechanismandlessonsfromgenetics
AT vinczemelinda inclusionbodymyositispathomechanismandlessonsfromgenetics
AT grigerzoltan inclusionbodymyositispathomechanismandlessonsfromgenetics
AT csonkatamas inclusionbodymyositispathomechanismandlessonsfromgenetics
AT szepesirita inclusionbodymyositispathomechanismandlessonsfromgenetics
AT kuruczandrea inclusionbodymyositispathomechanismandlessonsfromgenetics
AT dankokatalin inclusionbodymyositispathomechanismandlessonsfromgenetics
AT hortobagyitibor inclusionbodymyositispathomechanismandlessonsfromgenetics
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