Obscurins: Goliaths and Davids take over non-muscle tissues.

Obscurins comprise a family of proteins originally identified in striated muscles, where they play essential roles in myofibrillogenesis, cytoskeletal organization, and Ca(2+) homeostasis. They are encoded by the single OBSCN gene, and are composed of tandem adhesion domains and signaling motifs. To...

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Autores principales:	Maegen A Ackermann, Marey Shriver, Nicole A Perry, Li-Yen R Hu, Aikaterini Kontrogianni-Konstantopoulos
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2014
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/8e8df6fc9f4f4f4b915c4ca2432fea1c
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spelling	oai:doaj.org-article:8e8df6fc9f4f4f4b915c4ca2432fea1c2021-11-18T08:33:25ZObscurins: Goliaths and Davids take over non-muscle tissues.1932-620310.1371/journal.pone.0088162https://doaj.org/article/8e8df6fc9f4f4f4b915c4ca2432fea1c2014-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/24516603/?tool=EBIhttps://doaj.org/toc/1932-6203Obscurins comprise a family of proteins originally identified in striated muscles, where they play essential roles in myofibrillogenesis, cytoskeletal organization, and Ca(2+) homeostasis. They are encoded by the single OBSCN gene, and are composed of tandem adhesion domains and signaling motifs. To date, two giant obscurin isoforms have been described in detail that differ only at the extreme COOH-terminus; while obscurin-A (∼720 kDa) contains a non-modular COOH-terminus that harbors binding sites for the adaptor proteins ankyrins, obscurin-B (∼870 kDa) contains two COOH-terminal serine-threonine kinase domains preceded by adhesion motifs. Besides the two known giant obscurins, a thorough search of transcript databases suggests that complex alternative splicing of the obscurin transcript results in the generation of additional giant as well as small isoforms with molecular masses ranging between ∼50-970 kDa. These novel isoforms share common domains with the characterized isoforms, but also contain unique regions. Using a panel of highly specific antibodies directed against epitopes spanning the entire length of giant obscurins, we employed western blotting and immunohistochemistry to perform a systematic and comprehensive characterization of the expression profile of obscurins in muscle and non-muscle tissues. Our studies demonstrate for the first time that obscurins are not restricted to striated muscles, but are abundantly expressed in several tissues and organs including brain, skin, kidney, liver, spleen, and lung. While some obscurin isoforms are ubiquitously expressed, others are preferentially present in specific tissues and organs. Moreover, obscurins are present in select structures and cell types where they assume nuclear, cytosolic, and membrane distributions. Given the ubiquitous expression of some obscurins, along with the preferential expression of others, it becomes apparent that obscurins may play common and unique roles, respectively, in the regulation and maintenance of cell homeostasis in various tissues and organs throughout the body.Maegen A AckermannMarey ShriverNicole A PerryLi-Yen R HuAikaterini Kontrogianni-KonstantopoulosPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 9, Iss 2, p e88162 (2014)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Maegen A Ackermann Marey Shriver Nicole A Perry Li-Yen R Hu Aikaterini Kontrogianni-Konstantopoulos Obscurins: Goliaths and Davids take over non-muscle tissues.
description	Obscurins comprise a family of proteins originally identified in striated muscles, where they play essential roles in myofibrillogenesis, cytoskeletal organization, and Ca(2+) homeostasis. They are encoded by the single OBSCN gene, and are composed of tandem adhesion domains and signaling motifs. To date, two giant obscurin isoforms have been described in detail that differ only at the extreme COOH-terminus; while obscurin-A (∼720 kDa) contains a non-modular COOH-terminus that harbors binding sites for the adaptor proteins ankyrins, obscurin-B (∼870 kDa) contains two COOH-terminal serine-threonine kinase domains preceded by adhesion motifs. Besides the two known giant obscurins, a thorough search of transcript databases suggests that complex alternative splicing of the obscurin transcript results in the generation of additional giant as well as small isoforms with molecular masses ranging between ∼50-970 kDa. These novel isoforms share common domains with the characterized isoforms, but also contain unique regions. Using a panel of highly specific antibodies directed against epitopes spanning the entire length of giant obscurins, we employed western blotting and immunohistochemistry to perform a systematic and comprehensive characterization of the expression profile of obscurins in muscle and non-muscle tissues. Our studies demonstrate for the first time that obscurins are not restricted to striated muscles, but are abundantly expressed in several tissues and organs including brain, skin, kidney, liver, spleen, and lung. While some obscurin isoforms are ubiquitously expressed, others are preferentially present in specific tissues and organs. Moreover, obscurins are present in select structures and cell types where they assume nuclear, cytosolic, and membrane distributions. Given the ubiquitous expression of some obscurins, along with the preferential expression of others, it becomes apparent that obscurins may play common and unique roles, respectively, in the regulation and maintenance of cell homeostasis in various tissues and organs throughout the body.
format	article
author	Maegen A Ackermann Marey Shriver Nicole A Perry Li-Yen R Hu Aikaterini Kontrogianni-Konstantopoulos
author_facet	Maegen A Ackermann Marey Shriver Nicole A Perry Li-Yen R Hu Aikaterini Kontrogianni-Konstantopoulos
author_sort	Maegen A Ackermann
title	Obscurins: Goliaths and Davids take over non-muscle tissues.
title_short	Obscurins: Goliaths and Davids take over non-muscle tissues.
title_full	Obscurins: Goliaths and Davids take over non-muscle tissues.
title_fullStr	Obscurins: Goliaths and Davids take over non-muscle tissues.
title_full_unstemmed	Obscurins: Goliaths and Davids take over non-muscle tissues.
title_sort	obscurins: goliaths and davids take over non-muscle tissues.
publisher	Public Library of Science (PLoS)
publishDate	2014
url	https://doaj.org/article/8e8df6fc9f4f4f4b915c4ca2432fea1c
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Obscurins: Goliaths and Davids take over non-muscle tissues.

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