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Striated muscle preferentially expressed protein kinase

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https://www.readbyqxmd.com/read/27729468/striated-muscle-preferentially-expressed-protein-kinase-speg-is-essential-for-cardiac-function-by-regulating-junctional-membrane-complex-activity
#1
Ann P Quick, Qiongling Wang, Leonne E Philippen, Giselle Barreto-Torres, David Y Chiang, David L Beavers, Guoliang Wang, Maha Khalid, Julia O Reynolds, Hannah M Campbell, Jordan Showell, Mark D McCauley, Arjen Scholten, Xander H Wehrens
RATIONALE: Junctional membrane complexes (JMC) in myocytes are critical microdomains, in which excitation-contraction coupling occurs. Structural and functional disruption of JMCs underlies contractile dysfunction in failing hearts. However, the role of newly identified JMC protein striated muscle preferentially expressed gene (SPEG) remains unclear. OBJECTIVE: To determine the role of SPEG in healthy and failing adult hearts. MMethods and Results: Proteomic analysis of immunoprecipatated JMC-proteins ryanodine receptor type-2 (RyR2) and junctophilin-2 (JPH2) followed by mass spectrometry identified the serine-threonine kinase SPEG as the only novel binding partner for both proteins...
October 11, 2016: Circulation Research
https://www.readbyqxmd.com/read/25087613/speg-interacts-with-myotubularin-and-its-deficiency-causes-centronuclear-myopathy-with-dilated-cardiomyopathy
#2
Pankaj B Agrawal, Christopher R Pierson, Mugdha Joshi, Xiaoli Liu, Gianina Ravenscroft, Behzad Moghadaszadeh, Tiffany Talabere, Marissa Viola, Lindsay C Swanson, Göknur Haliloğlu, Beril Talim, Kyle S Yau, Richard J N Allcock, Nigel G Laing, Mark A Perrella, Alan H Beggs
Centronuclear myopathies (CNMs) are characterized by muscle weakness and increased numbers of central nuclei within myofibers. X-linked myotubular myopathy, the most common severe form of CNM, is caused by mutations in MTM1, encoding myotubularin (MTM1), a lipid phosphatase. To increase our understanding of MTM1 function, we conducted a yeast two-hybrid screen to identify MTM1-interacting proteins. Striated muscle preferentially expressed protein kinase (SPEG), the product of SPEG complex locus (SPEG), was identified as an MTM1-interacting protein, confirmed by immunoprecipitation and immunofluorescence studies...
August 7, 2014: American Journal of Human Genetics
https://www.readbyqxmd.com/read/24516603/obscurins-goliaths-and-davids-take-over-non-muscle-tissues
#3
Maegen A Ackermann, Marey Shriver, Nicole A Perry, Li-Yen R Hu, Aikaterini Kontrogianni-Konstantopoulos
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...
2014: PloS One
https://www.readbyqxmd.com/read/22951219/impact-of-titin-isoform-on-length-dependent-activation-and-cross-bridge-cycling-kinetics-in-rat-skeletal-muscle
#4
Ryan D Mateja, Marion L Greaser, Pieter P de Tombe
The magnitude of length dependent activation in striated muscle has been shown to vary with titin isoform. Recently, a rat that harbors a homozygous autosomal mutation (HM) causing preferential expression of a longer, giant titin isoform was discovered (Greaser et al. 2005). Here, we investigated the impact of titin isoform on myofilament force development and cross-bridge cycling kinetics as function of sarcomere length (SL) in tibialis anterior skeletal muscle isolated from wild type (WT) and HM. Skeletal muscle bundles from HM rats exhibited reductions in passive tension, maximal force development, myofilament calcium sensitivity, maximal ATP consumption, and tension cost at both short and long sarcomere length (SL=2...
April 2013: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/19118250/disruption-of-striated-preferentially-expressed-gene-locus-leads-to-dilated-cardiomyopathy-in-mice
#5
COMPARATIVE STUDY
Xiaoli Liu, Tripurasundari Ramjiganesh, Yen-Hsu Chen, Su Wol Chung, Sean R Hall, Scott L Schissel, Robert F Padera, Ronglih Liao, Kate G Ackerman, Jan Kajstura, Annarosa Leri, Piero Anversa, Shaw-Fang Yet, Matthew D Layne, Mark A Perrella
BACKGROUND: The striated preferentially expressed gene (Speg) generates 4 different isoforms through alternative promoter use and tissue-specific splicing. Depending on the cell type, Speg isoforms may serve as markers of striated or smooth muscle differentiation. METHODS AND RESULTS: To elucidate function of Speg gene isoforms, we disrupted the Speg gene locus in mice by replacing common exons 8, 9, and 10 with a lacZ gene. beta-Galactosidase activity was detected in cardiomyocytes of the developing heart starting at day 11...
January 20, 2009: Circulation
https://www.readbyqxmd.com/read/16545539/the-human-desmin-locus-gene-organization-and-lcr-mediated-transcriptional-control
#6
Jennifer L Y Tam, Kostas Triantaphyllopoulos, Helen Todd, Selina Raguz, Ton de Wit, Jennifer E Morgan, Terence A Partridge, Eleni Makrinou, Frank Grosveld, Michael Antoniou
Locus control regions (LCRs) are defined by their ability to confer reproducible physiological levels of transgene expression in mice and therefore thought to possess the ability to generate dominantly a transcriptionally active chromatin structure. We report the first characterization of a muscle-cell-specific LCR, which is linked to the human desmin gene (DES). The DES LCR consists of five regions of muscle-specific DNase I hypersensitivity (HS) localized between -9 and -18 kb 5' of DES and reproducibly drives full physiological levels of expression in all muscle cell types...
June 2006: Genomics
https://www.readbyqxmd.com/read/10973969/striated-muscle-preferentially-expressed-genes-alpha-and-beta-are-two-serine-threonine-protein-kinases-derived-from-the-same-gene-as-the-aortic-preferentially-expressed-gene-1
#7
C M Hsieh, S Fukumoto, M D Layne, K Maemura, H Charles, A Patel, M A Perrella, M E Lee
Aortic preferentially expressed gene (APEG)-1 is a 1.4-kilobase pair (kb) mRNA expressed in vascular smooth muscle cells and is down-regulated by vascular injury. An APEG-1 5'-end cDNA probe identified three additional isoforms. The 9-kb striated preferentially expressed gene (SPEG)alpha and the 11-kb SPEGbeta were found in skeletal muscle and heart. The 4-kb brain preferentially expressed gene was detected in the brain and aorta. We report here cloning of the 11-kb SPEGbeta cDNA. SPEGbeta encodes a 355-kDa protein that contains two serine/threonine kinase domains and is homologous to proteins of the myosin light chain kinase family...
November 24, 2000: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/9180014/myosin-isoforms-and-functional-diversity-in-vertebrate-smooth-muscle
#8
REVIEW
R A Murphy, J S Walker, J D Strauss
The expression of fast and slow myosin isoforms in individual cells is associated with differences in shortening velocities and power output in fully differentiated vertebrate striated muscle. This paradigm in which shortening velocity is determined by the myosin isoform (and load) is inappropriate for smooth muscle. Smooth muscle tissues express multiple myosin heavy and light chain isoforms, and it is not currently possible to separate and identify chemically distinct native myosin hexamers (i.e., isoforms)...
May 1997: Comparative Biochemistry and Physiology. Part B, Biochemistry & Molecular Biology
https://www.readbyqxmd.com/read/1717137/muscle-specific-gene-expression-in-rhabdomyosarcomas-and-stages-of-human-fetal-skeletal-muscle-development
#9
COMPARATIVE STUDY
P N Tonin, H Scrable, H Shimada, W K Cavenee
Rhabdomyosarcomas (RMS) bear a morphological resemblance to developing striated muscle. It has been reported that two histologically distinct subtypes of RMS, embryonal and alveolar, behave differently in many clinical aspects, such as age distribution, primary site, and prognosis. We have investigated the expression of various genes, which are preferentially expressed in normal muscle tissue or cell culture (actins, myosins, and creatine kinases, and myogenic regulatory genes MyoD, myogenin, MRF4, and Myf5), in embryonal and alveolar subtypes and compared the results to the stages of developing human fetal limb muscle...
October 1, 1991: Cancer Research
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