Myosin VI Localization and Expression in Striated Muscle Pathology

Justyna Karolczak; Serge Weis; Elisabeth Ehler; Biruta Kierdaszuk; Mariusz Berdynski; Cezary Zekanowski; Anna M. Kaminska; Maria Jolanta Redowicz

doi:10.1002/ar.22967

Myosin VI Localization and Expression in Striated Muscle Pathology

Justyna Karolczak, Serge Weis, Elisabeth Ehler, Biruta Kierdaszuk, Mariusz Berdynski, Cezary Zekanowski, Anna M. Kaminska, Maria Jolanta Redowicz^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

7 Citations (Scopus)

Abstract

Myosin VI (MVI) is a unique unconventional myosin translocating, unlike other myosins, towards the minus end of actin filaments. It is involved in numerous cellular processes such as endocytosis, intracellular trafficking, cell migration, and transcription. In mammalian skeletal muscles it localizes mainly to sarcoplasmic reticulum and is also present within the muscle nuclei and at the neuromuscular junction (Karolczak et al. Histochem Cell Biol 2013; 23:219-228). We have also shown that in denervated rat hindlimb muscle the MVI expression level is significantly increased and its localization is changed, indicating an important role of MVI in striated muscle pathology. Here, we addressed this problem by examining the distribution and expression levels of myosin VI in biopsies of skeletal muscles from patients with different myopathies. We found that, particularly in myopathies associated with fiber atrophy, the amount of MVI was enhanced and its localization in affected fibers was changed. Also, since a mutation within the human MVI gene was shown to be associated with cardiomyopathy, we assessed MVI localization and expression level in cardiac muscle using wild type and MLP(2/2) mice, a dilated cardiomyopathy model. No significant difference in MVI expression level was observed for both types of animals. MVI was found at intercalated discs and also at the sarcoplasmic reticulum. In the knockout mice, it was also present in ring-like structures surrounding the nuclei. The data indicate that in striated muscle MVI could be engaged in sarcoplasmic reticulum maintenance and/or functioning, vesicular transport, signal transmission and possibly in gene transcription.

Original language	English
Pages (from-to)	1706-1713
Number of pages	8
Journal	Anatomical record-Advances in integrative anatomy and evolutionary biology
Volume	297
Issue number	9
DOIs	https://doi.org/10.1002/ar.22967
Publication status	Published - Sept 2014

Keywords

myosin VI
unconventional myosin
muscle pathology
skeletal muscle
cardiac muscle
sarcoplasmic reticulum
SPINAL MUSCULAR-ATROPHY
BORDER CELL-MIGRATION
NEUROMUSCULAR-JUNCTION
SKELETAL-MUSCLE
PC12 CELLS
DEAFNESS
GENE
MICE
CARDIOMYOPATHY
AUTOPHAGY

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@article{48f8ce8fcf8543feb6d5f283a949dfe2,

title = "Myosin VI Localization and Expression in Striated Muscle Pathology",

abstract = "Myosin VI (MVI) is a unique unconventional myosin translocating, unlike other myosins, towards the minus end of actin filaments. It is involved in numerous cellular processes such as endocytosis, intracellular trafficking, cell migration, and transcription. In mammalian skeletal muscles it localizes mainly to sarcoplasmic reticulum and is also present within the muscle nuclei and at the neuromuscular junction (Karolczak et al. Histochem Cell Biol 2013; 23:219-228). We have also shown that in denervated rat hindlimb muscle the MVI expression level is significantly increased and its localization is changed, indicating an important role of MVI in striated muscle pathology. Here, we addressed this problem by examining the distribution and expression levels of myosin VI in biopsies of skeletal muscles from patients with different myopathies. We found that, particularly in myopathies associated with fiber atrophy, the amount of MVI was enhanced and its localization in affected fibers was changed. Also, since a mutation within the human MVI gene was shown to be associated with cardiomyopathy, we assessed MVI localization and expression level in cardiac muscle using wild type and MLP(2/2) mice, a dilated cardiomyopathy model. No significant difference in MVI expression level was observed for both types of animals. MVI was found at intercalated discs and also at the sarcoplasmic reticulum. In the knockout mice, it was also present in ring-like structures surrounding the nuclei. The data indicate that in striated muscle MVI could be engaged in sarcoplasmic reticulum maintenance and/or functioning, vesicular transport, signal transmission and possibly in gene transcription.",

keywords = "myosin VI, unconventional myosin, muscle pathology, skeletal muscle, cardiac muscle, sarcoplasmic reticulum, SPINAL MUSCULAR-ATROPHY, BORDER CELL-MIGRATION, NEUROMUSCULAR-JUNCTION, SKELETAL-MUSCLE, PC12 CELLS, DEAFNESS, GENE, MICE, CARDIOMYOPATHY, AUTOPHAGY",

author = "Justyna Karolczak and Serge Weis and Elisabeth Ehler and Biruta Kierdaszuk and Mariusz Berdynski and Cezary Zekanowski and Kaminska, {Anna M.} and Redowicz, {Maria Jolanta}",

year = "2014",

month = sep,

doi = "10.1002/ar.22967",

language = "English",

volume = "297",

pages = "1706--1713",

journal = "Anatomical record-Advances in integrative anatomy and evolutionary biology",

issn = "1932-8486",

publisher = "Wiley",

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TY - JOUR

T1 - Myosin VI Localization and Expression in Striated Muscle Pathology

AU - Karolczak, Justyna

AU - Weis, Serge

AU - Ehler, Elisabeth

AU - Kierdaszuk, Biruta

AU - Berdynski, Mariusz

AU - Zekanowski, Cezary

AU - Kaminska, Anna M.

AU - Redowicz, Maria Jolanta

PY - 2014/9

Y1 - 2014/9

N2 - Myosin VI (MVI) is a unique unconventional myosin translocating, unlike other myosins, towards the minus end of actin filaments. It is involved in numerous cellular processes such as endocytosis, intracellular trafficking, cell migration, and transcription. In mammalian skeletal muscles it localizes mainly to sarcoplasmic reticulum and is also present within the muscle nuclei and at the neuromuscular junction (Karolczak et al. Histochem Cell Biol 2013; 23:219-228). We have also shown that in denervated rat hindlimb muscle the MVI expression level is significantly increased and its localization is changed, indicating an important role of MVI in striated muscle pathology. Here, we addressed this problem by examining the distribution and expression levels of myosin VI in biopsies of skeletal muscles from patients with different myopathies. We found that, particularly in myopathies associated with fiber atrophy, the amount of MVI was enhanced and its localization in affected fibers was changed. Also, since a mutation within the human MVI gene was shown to be associated with cardiomyopathy, we assessed MVI localization and expression level in cardiac muscle using wild type and MLP(2/2) mice, a dilated cardiomyopathy model. No significant difference in MVI expression level was observed for both types of animals. MVI was found at intercalated discs and also at the sarcoplasmic reticulum. In the knockout mice, it was also present in ring-like structures surrounding the nuclei. The data indicate that in striated muscle MVI could be engaged in sarcoplasmic reticulum maintenance and/or functioning, vesicular transport, signal transmission and possibly in gene transcription.

AB - Myosin VI (MVI) is a unique unconventional myosin translocating, unlike other myosins, towards the minus end of actin filaments. It is involved in numerous cellular processes such as endocytosis, intracellular trafficking, cell migration, and transcription. In mammalian skeletal muscles it localizes mainly to sarcoplasmic reticulum and is also present within the muscle nuclei and at the neuromuscular junction (Karolczak et al. Histochem Cell Biol 2013; 23:219-228). We have also shown that in denervated rat hindlimb muscle the MVI expression level is significantly increased and its localization is changed, indicating an important role of MVI in striated muscle pathology. Here, we addressed this problem by examining the distribution and expression levels of myosin VI in biopsies of skeletal muscles from patients with different myopathies. We found that, particularly in myopathies associated with fiber atrophy, the amount of MVI was enhanced and its localization in affected fibers was changed. Also, since a mutation within the human MVI gene was shown to be associated with cardiomyopathy, we assessed MVI localization and expression level in cardiac muscle using wild type and MLP(2/2) mice, a dilated cardiomyopathy model. No significant difference in MVI expression level was observed for both types of animals. MVI was found at intercalated discs and also at the sarcoplasmic reticulum. In the knockout mice, it was also present in ring-like structures surrounding the nuclei. The data indicate that in striated muscle MVI could be engaged in sarcoplasmic reticulum maintenance and/or functioning, vesicular transport, signal transmission and possibly in gene transcription.

KW - myosin VI

KW - unconventional myosin

KW - muscle pathology

KW - skeletal muscle

KW - cardiac muscle

KW - sarcoplasmic reticulum

KW - SPINAL MUSCULAR-ATROPHY

KW - BORDER CELL-MIGRATION

KW - NEUROMUSCULAR-JUNCTION

KW - SKELETAL-MUSCLE

KW - PC12 CELLS

KW - DEAFNESS

KW - GENE

KW - MICE

KW - CARDIOMYOPATHY

KW - AUTOPHAGY

U2 - 10.1002/ar.22967

DO - 10.1002/ar.22967

M3 - Article

SN - 1932-8486

VL - 297

SP - 1706

EP - 1713

JO - Anatomical record-Advances in integrative anatomy and evolutionary biology

JF - Anatomical record-Advances in integrative anatomy and evolutionary biology

IS - 9

ER -

Myosin VI Localization and Expression in Striated Muscle Pathology

Abstract

Keywords

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