Cytoplasmic body pathology in severe ACTA1-myopathy in the absence of typical nemaline-rod histology

2015 ◽  
Vol 25 ◽  
pp. S286-S287
Author(s):  
S. Donkervoort ◽  
S. Chan ◽  
N. Bradley ◽  
A. Foley ◽  
D. Nguyen ◽  
...  
Keyword(s):  
2010 ◽  
Vol 285 (45) ◽  
pp. 34508-34517 ◽  
Author(s):  
Seiga Ohmine ◽  
Ryuta Sakuma ◽  
Toshie Sakuma ◽  
Tayaramma Thatava ◽  
Gonzalo P. Solis ◽  
...  

2008 ◽  
Vol 82 (13) ◽  
pp. 6395-6408 ◽  
Author(s):  
Leslie J. Marshall ◽  
Amy C. Moore ◽  
Misao Ohki ◽  
Issay Kitabayashi ◽  
David Patterson ◽  
...  

ABSTRACT The localization of the adenovirus E1B-55K-E4orf6 protein complex is critical for its function. Prior studies demonstrated that E4orf6 directs the nuclear localization of E1B-55K in human cells and in rodent cells that contain part of human chromosome 21. We show here that the relevant activity on chromosome 21 maps to RUNX1. RUNX1 proteins are transcription factors that serve as scaffolds for the assembly of proteins that regulate transcription and RNA processing. After transfection, the RUNX1a, RUNX1b, and RUNX1-ΔN variants allowed E4orf6-directed E1B-55K nuclear localization. The failure of RUNX1c to allow nuclear colocalization was relieved by the deletion of amino-terminal residues of this protein. In the adenovirus-infected mouse cell, RUNX1 proteins were localized to discrete structures about the periphery of viral replication centers. These sites are enriched in viral RNA and RNA-processing factors. RUNX1b and RUNX1a proteins displaced E4orf6 from these sites. The association of E1B-55K at viral replication centers was enhanced by the RUNX1a and RUNX1b proteins, but only in the absence of E4orf6. In the presence of E4orf6, E1B-55K occurred in a perinuclear cytoplasmic body resembling the aggresome and was excluded from the nucleus of the infected mouse cell. We interpret these findings to mean that a dynamic relationship exists between the E4orf6, E1B-55K, and RUNX1 proteins. In cooperation with E4orf6, RUNX1 proteins are able to modulate the localization of E1B-55K and even remodel virus-specific structures that form at late times of infection. Subsequent studies will need to determine a functional consequence of the interaction between E4orf6, E1B-55K, and RUNX1.


PLoS ONE ◽  
2017 ◽  
Vol 12 (1) ◽  
pp. e0169436 ◽  
Author(s):  
Yuki Kawaguchi ◽  
Masato Taoka ◽  
Takahiro Takekiyo ◽  
Takamasa Uekita ◽  
Ikuo Shoji ◽  
...  

1990 ◽  
Vol 99 (2-3) ◽  
pp. 291-300 ◽  
Author(s):  
Ko Sahashi ◽  
Kinji Ohno ◽  
Masashi Tanaka ◽  
Tohru Ibi ◽  
Tomoko Yamamoto ◽  
...  

PLoS ONE ◽  
2015 ◽  
Vol 10 (3) ◽  
pp. e0121666 ◽  
Author(s):  
Feng Liang Liu ◽  
Yi Qun Kuang ◽  
Dan Mu ◽  
Hong Yi Zheng ◽  
Jia Wu Zhu ◽  
...  

1993 ◽  
Vol 170 (4) ◽  
pp. 261-272 ◽  
Author(s):  
KOJI ABE ◽  
KAZUO KOBAYASHI ◽  
KEIJI CHIDA ◽  
NORIKO KIMURA ◽  
KYUYA KOGURE
Keyword(s):  

Neurology ◽  
1998 ◽  
Vol 51 (3) ◽  
pp. 873-875 ◽  
Author(s):  
J. Vajsar ◽  
T. Balslev ◽  
P. N. Ray ◽  
J. Siegel-Bartelt ◽  
V. Jay

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