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Journal articles on the topic 'Fused in Sarcoma'

Author: Grafiati

Published: 4 June 2021

Last updated: 13 February 2022

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1

Mackenzie, Ian R. A., and Manuela Neumann. "Fused in Sarcoma Neuropathology in Neurodegenerative Disease." Cold Spring Harbor Perspectives in Medicine 7, no. 12 (January 17, 2017): a024299. http://dx.doi.org/10.1101/cshperspect.a024299.

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2

Underwood, Caroline I. M., Diana M. Cardona, Rex C. Bentley, Guomiao Shen, Xiaojun Feng, George Jour, and Rami N. Al-Rohil. "Epithelioid Hyalinizing Sarcoma With MGA-NUTM1 Fusion." American Journal of Clinical Pathology 154, no. 6 (September 3, 2020): 859–66. http://dx.doi.org/10.1093/ajcp/aqaa113.

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Abstract Objectives Soft tissue sarcomas are a group of tumors derived from the mesenchymal origin. Historically, they have been classified according to morphologic and immunohistochemical characteristics. The advent of multiplexed next-generation sequencing (NGS), specifically RNA sequencing, has modified the classification of such tumors and others by determining categorization based on molecular alterations. The NUTM1 rearrangement, previously thought to be present only in carcinomas, has recently been reported in poorly differentiated high-grade sarcomas of the soft tissue. We present the first reported case of an epithelioid hyalinizing sarcoma harboring the MGA-NUTM1 fusion in an acral site. Methods Histopathologic, immunohistochemical, and molecular testing were performed on resection tissue. Results Histologically, the tumor showed an epithelioid morphology with prominent background hyalinization. Immunohistochemically, the tumor expressed CD99 and nuclear NUT-1. By NGS the tumor harbors MGA-NUTM1 fusion. Conclusions Our findings support more extensive use of NGS for accurate sarcoma classification and identification of potential therapeutic targets. Furthermore, they corroborate the fact that NUTM1-rearranged soft tissue tumors represent a spectrum of heterogeneous morphologic entities. This case also highlights the utility of NUT-1 immunohistochemical study as a possible screening tool for NUTM1-fused sarcomas.

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3

Matus, Soledad, Daryl A. Bosco, and Claudio Hetz. "Autophagy meets fused in sarcoma-positive stress granules." Neurobiology of Aging 35, no. 12 (December 2014): 2832–35. http://dx.doi.org/10.1016/j.neurobiolaging.2014.08.019.

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4

Siozopoulou, Vasiliki, Evelien Smits, Koen De Winne, Elly Marcq, and Patrick Pauwels. "NTRK Fusions in Sarcomas: Diagnostic Challenges and Clinical Aspects." Diagnostics 11, no. 3 (March 9, 2021): 478. http://dx.doi.org/10.3390/diagnostics11030478.

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Tropomyosin receptor kinase (TK) is encoded by the neurotrophic tyrosine receptor kinase genes (NTRK) 1, 2, and 3, whose activation plays an important role in cell cycle proliferation and survival. Fusions of one of these genes can lead to constitutive activation of TRK, which can potentially be oncogenic. NTRK fusions are commonly present in rare histologic tumor types. Among sarcomas, infantile fibrosarcoma shows NTRK fusion in more than 90% of the cases. Many other sarcoma types are also investigated for NTRK fusions. These fusions are druggable alteration of the agnostic type, meaning that all NTRK fused tumors can be treated with NTRK-inhibitors regardless of tumor type or tissue of origin. TRK-inhibitors have shown good response rates, with durable effects and limited side effects. Resistance to therapy will eventually occur in some cases, wherefore the next-generation TRK-inhibitors are introduced. The diagnosis of NTRK fused tumors, among them sarcomas, remains an issue, as many algorithms but no guidelines exist to date. Given the importance of this diagnosis, in this paper we aim to (1) analyze the histopathological features of sarcomas that correlate more often with NTRK fusions, (2) give an overview of the TRK-inhibitors and the problems that arise from resistance to the therapy, and (3) discuss the diagnostic algorithms of NTRK fused tumors with emphasis on sarcomas.

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5

Jia, Weiyan, Sang Hwa Kim, Mark A. Scalf, Peter Tonzi, Robert J. Millikin, William M. Guns, Lu Liu, et al. "Fused in sarcoma regulates DNA replication timing and kinetics." Journal of Biological Chemistry 297, no. 3 (September 2021): 101049. http://dx.doi.org/10.1016/j.jbc.2021.101049.

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6

Dormann, Dorothee, and Christian Haass. "Fused in sarcoma (FUS): An oncogene goes awry in neurodegeneration." Molecular and Cellular Neuroscience 56 (September 2013): 475–86. http://dx.doi.org/10.1016/j.mcn.2013.03.006.

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7

Orozco, Denise, Sabina Tahirovic, Kristin Rentzsch, Benjamin M. Schwenk, Christian Haass, and Dieter Edbauer. "Loss of fused in sarcoma (FUS) promotes pathological Tau splicing." EMBO reports 13, no. 8 (June 19, 2012): 759–64. http://dx.doi.org/10.1038/embor.2012.90.

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8

McAninch, Damian, and Mihaela-Rita Mihailescu. "Fused in Sarcoma (FUS) Targets Neuronal G Quadruplex Containing mRNAS." Biophysical Journal 110, no. 3 (February 2016): 240a. http://dx.doi.org/10.1016/j.bpj.2015.11.1322.

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9

Chen, Chen, Xiufang Ding, Nimrah Akram, Song Xue, and Shi-Zhong Luo. "Fused in Sarcoma: Properties, Self-Assembly and Correlation with Neurodegenerative Diseases." Molecules 24, no. 8 (April 24, 2019): 1622. http://dx.doi.org/10.3390/molecules24081622.

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Fused in sarcoma (FUS) is a DNA/RNA binding protein that is involved in RNA metabolism and DNA repair. Numerous reports have demonstrated by pathological and genetic analysis that FUS is associated with a variety of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration (FTLD), and polyglutamine diseases. Traditionally, the fibrillar aggregation of FUS was considered to be the cause of those diseases, especially via its prion-like domains (PrLDs), which are rich in glutamine and asparagine residues. Lately, a nonfibrillar self-assembling phenomenon, liquid–liquid phase separation (LLPS), was observed in FUS, and studies of its functions, mechanism, and mutual transformation with pathogenic amyloid have been emerging. This review summarizes recent studies on FUS self-assembling, including both aggregation and LLPS as well as their relationship with the pathology of ALS, FTLD, and other neurodegenerative diseases.

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10

Kakushkin, N. "A. A. Muratov. - To the question of sarcoma transplantation to the healthy part of the body in the same patient. (Yezhenedelnik, 1895, No. 15)." Journal of obstetrics and women's diseases 9, no. 7-8 (October 22, 2020): 659. http://dx.doi.org/10.17816/jowd97-8659.

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Cases of self-infection with cancer are described by Sippel. The author describes a case of self-infection with sarcoma. Dressmaker, 14 years old; complaints of severe constant pain in the lower abdomen. Recognized the neoplasm of common Fallopian tubes, or ovaries, probably of a sarcomatous nature. Gluttony showed that the tumor, extensively fused with the surrounding organs, belongs to the right appendage. When it was separated, part of its clumpy-purulent contents poured out into the abdominal cavity. The entire tumor was removed and, carefully examined, turned out to be a round-large-cell sarcoma, containing parts of fusiform cells in places.

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11

Rutherford, Nicola J., Nicole A. Finch, Mariely DeJesus-Hernandez, Richard J. P. Crook, Catherine Lomen-Hoerth, Zbigniew K. Wszolek, Ryan J. Uitti, Neill R. Graff-Radford, and Rosa Rademakers. "Pathogenicity of exonic indels in fused in sarcoma in amyotrophic lateral sclerosis." Neurobiology of Aging 33, no. 2 (February 2012): 424.e23–424.e24. http://dx.doi.org/10.1016/j.neurobiolaging.2010.09.029.

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12

Soo, K. Y., and J. D. Atkin. "Autophagy dysregulation by mutant fused in sarcoma—implications for amyotrophic lateral sclerosis." Cell Death & Disease 6, no. 10 (October 2015): e1945-e1945. http://dx.doi.org/10.1038/cddis.2015.311.

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13

Dormann, Dorothee, Ramona Rodde, Dieter Edbauer, Eva Bentmann, Ingeborg Fischer, Alexander Hruscha, Manuel E. Than, et al. "ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import." EMBO Journal 29, no. 16 (July 6, 2010): 2841–57. http://dx.doi.org/10.1038/emboj.2010.143.

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14

Yang, Shu, Sadaf T. Warraich, Garth A. Nicholson, and Ian P. Blair. "Fused in sarcoma/translocated in liposarcoma: A multifunctional DNA/RNA binding protein." International Journal of Biochemistry & Cell Biology 42, no. 9 (September 2010): 1408–11. http://dx.doi.org/10.1016/j.biocel.2010.06.003.

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15

Shalaby, A., A. Alhussain, Y. Al Hmada, and A. Bernieh. "A Case Report Of A Spindle Cell Sarcoma With Ntrk-3 Rearrangement And A Novel Gene Fusion Partner (Ntrk3-Eif2s2)." American Journal of Clinical Pathology 154, Supplement_1 (October 2020): S69—S70. http://dx.doi.org/10.1093/ajcp/aqaa161.151.

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Abstract Casestudy A group of spindle cell tumors with characteristic morphologic features, co-expression of CD34 and S100 and recurrent gene rearrangements in RAF-1, BRAF, and NTRK has recently been described. These tumors were found to be previously labeled as malignant peripheral nerve sheath tumor, infantile fibrosarcoma or unclassified spindle cell sarcoma. We describe a case of a 25-year-old female who presented with a right thigh mass. She underwent an ultrasound-guided biopsy showing a spindle cell tumor with co-expression of CD34 and S100 and subsequently underwent resection of the mass. Gross examination showed a 7.5 cm multi-lobulated, tan-pink, hemorrhagic and fleshy mass. Histologically, the tumor was relatively well-demarcated and consisted of spindle cells with moderate to high cellularity in a patternless architecture. The spindle cells showed moderate to marked pleomorphism, pale amphophilic cytoplasm, ovoid-to-elongated nuclei with vesicular chromatin, and occasional prominent nucleoli. Areas of prominent perivascular and stromal hyalinization were seen. Mitotic activity was brisk with up to 33 mitoses per 10 high power fields. Necrosis representing approximately 5% of the mass was identified. On immunohistochemistry, the tumor cells showed strong and diffuse positivity for CD34 and S100 and were negative for SOX10, broad-spectrum cytokeratin, EMA, SMA, Desmin, STAT6, MUC4, TLE1, and H3K27me3 (retained nuclear expression). EIF2S2-NTRK3 fusion gene was detected using next generation sequencing analysis. Conclusion A few cases of NTRK3 spindle cell sarcomas, other than classic infantile fibrosarcoma, have been previously reported in the literature with fusion genes involving ETV6, EML4, and STRN, among others. A gene fusion involving NTRK3 and EIF2S2 has not been previously reported. NTRK3-fused sarcomas typically show high-grade morphology and aggressive clinical behavior. Identification of NTRK-fused sarcomas is clinically important, as these advanced tumors are potentially amenable to NTRK inhibition. In our case, patient received adjuvant post-operative radiation therapy and returned with lung metastasis 5 months after surgery.

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16

Dhar, Sanjit Kumar, Jiayu Zhang, Jozsef Gal, Yong Xu, Lu Miao, Bert C. Lynn, Haining Zhu, Edward J. Kasarskis, and Daret K. St. Clair. "FUsed in Sarcoma Is a Novel Regulator of Manganese Superoxide Dismutase Gene Transcription." Antioxidants & Redox Signaling 20, no. 10 (April 2014): 1550–66. http://dx.doi.org/10.1089/ars.2012.4984.

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17

Cha, S. J., H. ‐J Choi, H. ‐J Kim, E. J. Choi, K. ‐H Song, D. S. Im, and K. Kim. "Parkin expression reverses mitochondrial dysfunction in fused in sarcoma‐induced amyotrophic lateral sclerosis." Insect Molecular Biology 29, no. 1 (July 12, 2019): 56–65. http://dx.doi.org/10.1111/imb.12608.

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18

Lashley, T., J. D. Rohrer, R. Bandopadhyay, C. Fry, Z. Ahmed, A. M. Isaacs, J. H. Brelstaff, et al. "A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathies." Brain 134, no. 9 (July 12, 2011): 2548–64. http://dx.doi.org/10.1093/brain/awr160.

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19

Ge, Zhouhong, Zhuoan Cheng, Xinrong Yang, Xisong Huo, Ning Wang, Hui Wang, Cun Wang, et al. "Long noncoding RNASchLAHsuppresses metastasis of hepatocellular carcinoma through interacting with fused in sarcoma." Cancer Science 108, no. 4 (April 2017): 653–62. http://dx.doi.org/10.1111/cas.13200.

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20

Ishikawa, F., F. Takaku, M. Nagao, and T. Sugimura. "Rat c-raf oncogene activation by a rearrangement that produces a fused protein." Molecular and Cellular Biology 7, no. 3 (March 1987): 1226–32. http://dx.doi.org/10.1128/mcb.7.3.1226.

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In a previous study, activated rat c-raf was detected by an NIH 3T3 cell transfection assay, and a rearrangement was demonstrated in the 5' half of the sequence of the gene. In the present study, the cDNAs of normal and activated rat c-raf were analyzed. Results showed that the activated c-raf gene is transcribed to produce a fused mRNA, in which the 5' half of the sequence is replaced by an unknown rat sequence. This mRNA codes a fused c-raf protein. The normal and activated c-raf cDNAs were each connected to the long terminal repeat of Rous sarcoma virus and transfected into NIH 3T3 cells. Only the activated form had transforming activity. We conclude that the rearrangement is responsible for the activation of c-raf.

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21

Ishikawa, F., F. Takaku, M. Nagao, and T. Sugimura. "Rat c-raf oncogene activation by a rearrangement that produces a fused protein." Molecular and Cellular Biology 7, no. 3 (March 1987): 1226–32. http://dx.doi.org/10.1128/mcb.7.3.1226-1232.1987.

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In a previous study, activated rat c-raf was detected by an NIH 3T3 cell transfection assay, and a rearrangement was demonstrated in the 5' half of the sequence of the gene. In the present study, the cDNAs of normal and activated rat c-raf were analyzed. Results showed that the activated c-raf gene is transcribed to produce a fused mRNA, in which the 5' half of the sequence is replaced by an unknown rat sequence. This mRNA codes a fused c-raf protein. The normal and activated c-raf cDNAs were each connected to the long terminal repeat of Rous sarcoma virus and transfected into NIH 3T3 cells. Only the activated form had transforming activity. We conclude that the rearrangement is responsible for the activation of c-raf.

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22

Ryu, Hyun-Hee, Mi-Hee Jun, Kyung-Jin Min, Deok-Jin Jang, Yong-Seok Lee, Hyong Kyu Kim, and Jin-A. Lee. "Autophagy regulates amyotrophic lateral sclerosis-linked fused in sarcoma-positive stress granules in neurons." Neurobiology of Aging 35, no. 12 (December 2014): 2822–31. http://dx.doi.org/10.1016/j.neurobiolaging.2014.07.026.

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23

Jun, Mi-Hee, and Jin-A. Lee. "Analysis of domain required for aggregates formation of ALS (Amyotrophic lateral sclerosis)/FTD (Frontotemporal dementia)-linked FUS in mammalian cells." Analytical Science and Technology 28, no. 5 (October 25, 2015): 331–40. http://dx.doi.org/10.5806/ast.2015.28.5.331.

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24

Rovnak, Joel, and Sandra L. Quackenbush. "Walleye Dermal Sarcoma Virus Retroviral Cyclin Directly Contacts TAF9." Journal of Virology 80, no. 24 (October 11, 2006): 12041–48. http://dx.doi.org/10.1128/jvi.01425-06.

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ABSTRACT Walleye dermal sarcoma virus (WDSV) is a complex retrovirus associated with dermal sarcomas in walleye fish. A WDSV accessory gene encodes a cyclin homolog or retroviral cyclin (rv-cyclin). WDSV rv-cyclin was found to be associated with transcription complexes and to affect transcription in a cell-type and promoter-dependent manner. It inhibited the WDSV promoter in walleye fibroblasts and activated transcription from GAL4 promoters when fused to the GAL4 DNA binding domain, and an activation domain (AD) has been localized to 30 amino acids in the carboxyl region. rv-cyclin can block the pulldown of transcription coactivators by the AD of VP16, and the isolated rv-cyclin AD interferes specifically with the interaction between the carboxyl halves of the VP16 AD, VP16C, and TATA-binding protein-associated factor 9 (TAF9). The carboxyl region and isolated AD can bind TAF9 directly in assays of protein-protein interaction in vitro. Furthermore, rv-cyclin and the isolated rv-cyclin AD interfere specifically with the function of VP16C in transcription assays. A previously identified motif within the VP16C sequence mediates TAF9 binding, and this motif is present in the activation domains of a variety of TAF9-binding transcriptional activators. A similar motif is present in the rv-cyclin AD, and point mutations within this motif affect rv-cyclin function and protein-protein interactions. The results support a model of transcription regulation by direct interaction with TAF9.

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25

Stronati, Eleonora, Stefano Biagioni, Mario Fiore, Mauro Giorgi, Giancarlo Poiana, Camilla Toselli, and Emanuele Cacci. "Wild-Type and Mutant FUS Expression Reduce Proliferation and Neuronal Differentiation Properties of Neural Stem Progenitor Cells." International Journal of Molecular Sciences 22, no. 14 (July 15, 2021): 7566. http://dx.doi.org/10.3390/ijms22147566.

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Nervous system development involves proliferation and cell specification of progenitor cells into neurons and glial cells. Unveiling how this complex process is orchestrated under physiological conditions and deciphering the molecular and cellular changes leading to neurological diseases is mandatory. To date, great efforts have been aimed at identifying gene mutations associated with many neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Mutations in the RNA/DNA binding protein Fused in Sarcoma/Translocated in Liposarcoma (FUS/TLS) have been associated with motor neuron degeneration in rodents and humans. Furthermore, increased levels of the wild-type protein can promote neuronal cell death. Despite the well-established causal link between FUS mutations and ALS, its role in neural cells remains elusive. In order to shed new light on FUS functions we studied its role in the control of neural stem progenitor cell (NSPC) properties. Here, we report that human wild-type Fused in Sarcoma (WT FUS), exogenously expressed in mouse embryonic spinal cord-derived NSPCs, was localized in the nucleus, caused cell cycle arrest in G1 phase by affecting cell cycle regulator expression, and strongly reduced neuronal differentiation. Furthermore, the expression of the human mutant form of FUS (P525L-FUS), associated with early-onset ALS, drives the cells preferentially towards a glial lineage, strongly reducing the number of developing neurons. These results provide insight into the involvement of FUS in NSPC proliferation and differentiation into neurons and glia.

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26

Gao, Kai, Wen Zheng, Xiong Deng, Wei Xiong, Zhi Song, Yan Yang, and Hao Deng. "Genetic analysis of the fused in sarcoma gene in Chinese Han patients with Parkinson's disease." Parkinsonism & Related Disorders 20, no. 1 (January 2014): 119–21. http://dx.doi.org/10.1016/j.parkreldis.2013.09.010.

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27

Zheng, Wen, Xiong Deng, Hui Liang, Zhi Song, Kai Gao, Yan Yang, and Hao Deng. "Genetic analysis of the fused in sarcoma gene in Chinese Han patients with essential tremor." Neurobiology of Aging 34, no. 8 (August 2013): 2078.e3–2078.e4. http://dx.doi.org/10.1016/j.neurobiolaging.2013.03.001.

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28

Flies, C. M., and J. H. Veldink. "Chorea is a pleiotropic clinical feature of mutated fused-in-sarcoma in amyotrophic lateral sclerosis." Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 21, no. 3-4 (March 2, 2020): 309–11. http://dx.doi.org/10.1080/21678421.2020.1733021.

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29

Hartikainen, Päivi H., Maria Pikkarainen, Tuomo Hänninen, Hilkka Soininen, and Irina Alafuzoff. "Unusual clinical presentation and neuropathology in two subjects with fused-in sarcoma (FUS) positive inclusions." Neuropathology 32, no. 1 (April 26, 2011): 60–68. http://dx.doi.org/10.1111/j.1440-1789.2011.01218.x.

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30

Loy, Clement T., Elizabeth McCusker, Jillian J. Kril, John B. Kwok, William S. Brooks, Heather McCann, Adrian M. Isaacs, and Glenda M. Halliday. "Very early-onset frontotemporal dementia with no family history predicts underlying fused in sarcoma pathology." Brain 133, no. 12 (August 7, 2010): e158-e158. http://dx.doi.org/10.1093/brain/awq186.

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31

Shiihashi, Gen, Daisuke Ito, Itaru Arai, Yuki Kobayashi, Kanehiro Hayashi, Shintaro Otsuka, Kazunori Nakajima, Michisuke Yuzaki, Shigeyoshi Itohara, and Norihiro Suzuki. "Dendritic Homeostasis Disruption in a Novel Frontotemporal Dementia Mouse Model Expressing Cytoplasmic Fused in Sarcoma." EBioMedicine 24 (October 2017): 102–15. http://dx.doi.org/10.1016/j.ebiom.2017.09.005.

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32

Aoki, Naoya, Shinji Higashi, Ito Kawakami, Zen Kobayashi, Masato Hosokawa, Omi Katsuse, Takashi Togo, Yoshio Hirayasu, and Haruhiko Akiyama. "Localization of fused in sarcoma (FUS) protein to the post-synaptic density in the brain." Acta Neuropathologica 124, no. 3 (April 18, 2012): 383–94. http://dx.doi.org/10.1007/s00401-012-0984-6.

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33

Sagaert, Xavier, Eric Van Cutsem, Sabine Tejpar, Hans Prenen, Gert De Hertogh, Philippe Nafteux, Karin Haustermans, and Maria Debiec-Rychter. "Upper gastrointestinal cancers: Are all carcinomas truly carcinomas?" Journal of Clinical Oncology 32, no. 3_suppl (January 20, 2014): 20. http://dx.doi.org/10.1200/jco.2014.32.3_suppl.20.

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20 Background: Poorly differentiated cancer of the upper GI tract is a challenging diagnosis for the pathologist, who will rely on a positive cytokeratin immunostaining to confirm the epithelial origin of the tumor. Being confronted with a highly unusual case (a poorly differentiated esophageal “carcinoma” with immunophenotypic and genetic sarcoma-like features, arising in the background of high grade Barrett dysplasia), we retrospectively investigated whether other cancer cases displayed the same unusual features. Methods: 34 poorly differentiated GI cancers were selected based on availability of FFPE. All cases were investigated by (1) immunohistochemistry for selected carcinoma and sarcoma markers (prekeratin, NCAM, vimentin, CD99) and by (2) FISH for presence/absence of abnormalities of 2 genes, SYT and EWS, that are frequently rearranged in synovial and Ewing’s sarcoma respectively. Results: 6/34 cases displayed an aberrant immunophenotype, with expression of NCAM, vimentin, and/or CD99. FISH revealed a substantial number (9/34) of SYT and EWS related anomalies: 1 EWS rearrangements, 1 SYT rearrangements, 1 SYT and 1 EWS focal amplification, and 4 aneuploidies. While EWS and SYT are (mostly) fused to ERG or SSX1/2 in Ewing’s and synovial sarcoma respectively, these genes were found to be intact in our EWS and SYT rearranged cases. Conclusions: Our data show that SYT & EWS abnormalities are recurrently occurring in poorly differentiated GI cancers that are (mis?)diagnosed as carcinomas. While EWS rearrangement has previously been described in cancer types other than Ewing’s sarcoma, SYT rearrangement was so far thought to be exclusive to the diagnosis of synovial sarcoma. In addition, EWS/SYT abnormalities in our series seemed to target cancers that are at risk of not responding well to the conventional chemoradiotherapy regimen. Of interest, the one “carcinoma” patient that started this study went into complete remission after being administered a sarcoma targeted therapy. We also infer from our results that good histologic assessment of GI cancers is essential for clinical trials aiming at targeted therapy, as not all carcinomas may present true carcinomas.

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34

Bradfield, Nicholas I., Catriona McLean, John Drago, David G. Darby, and David Ames. "Rapidly progressive Fronto-temporal dementia (FTD) associated with Frontotemporal lobar degeneration (FTLD) in the presence of Fused in Sarcoma (FUS) protein: a rare, sporadic, and aggressive form of FTD." International Psychogeriatrics 29, no. 10 (June 29, 2017): 1743–46. http://dx.doi.org/10.1017/s1041610217001193.

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ABSTRACTFronto-temporal dementia (FTD) associated with Fused in Sarcoma (FUS) protein accumulation is an uncommon cause of FTD with a distinct syndrome of young age onset behavioral variant FTD, without a family history of FTD and caudate atrophy. We present a sporadic case of a 61-year-old patient with mixed features of both behavioral variant FTD with later semantic language dissolution associated with pathologically proven FUS. He was older than usual for FUS pathology, his course was rapidly progressive, and he had atypical language features. This case broadens the clinical spectrum caused by FUS-protein-related FTD.

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35

Aoki, Masashi. "Amyotrophic lateral sclerosis (ALS) with the mutations in the fused in sarcoma/translocated in liposarcoma gene." Rinsho Shinkeigaku 53, no. 11 (2013): 1080–83. http://dx.doi.org/10.5692/clinicalneurol.53.1080.

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36

Ortega-Cubero, S., O. Lorenzo-Betancor, E. Lorenzo, E. Alonso, F. Coria, M. A. Pastor, R. Fernández-Santiago, et al. "Fused in Sarcoma (FUS) gene mutations are not a frequent cause of essential tremor in Europeans." Neurobiology of Aging 34, no. 10 (October 2013): 2441.e9–2441.e11. http://dx.doi.org/10.1016/j.neurobiolaging.2013.04.024.

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37

Asai, K., H. Sumi-Akamaru, A. Nishikawa, E. Shirahata, R. Yamashita, N. Miyashita, H. Mochizuki, and T. Naka. "Fused in sarcoma (FUS) pathology observed in an autopsy case of ALS/MND-plus clinical syndrome." Journal of the Neurological Sciences 405 (October 2019): 339. http://dx.doi.org/10.1016/j.jns.2019.10.1468.

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38

Neumann, Manuela. "Reply: Very early-onset frontotemporal dementia with no family history predicts underlying fused in sarcoma pathology." Brain 133, no. 12 (August 9, 2010): e159-e159. http://dx.doi.org/10.1093/brain/awq189.

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39

Bronisz, Agnieszka, Heather A. Carey, Jakub Godlewski, Said Sif, Michael C. Ostrowski, and Sudarshana M. Sharma. "The Multifunctional Protein Fused in Sarcoma (FUS) Is a Coactivator of Microphthalmia-associated Transcription Factor (MITF)." Journal of Biological Chemistry 289, no. 1 (November 20, 2013): 326–34. http://dx.doi.org/10.1074/jbc.m113.493874.

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40

Haass, Christian, Ramona Rodde, Dieter Edbauer, Eva Bentmann, Ingeborg Fischer, Alexander Hruscha, Manuel Than, et al. "O4-03-06: Familial Als-associated Fused in Sarcoma (fus) Mutations Disrupt Transportin-mediated Nuclear Import." Alzheimer's & Dementia 6 (July 2010): e17-e17. http://dx.doi.org/10.1016/j.jalz.2010.08.051.

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Koustas, Evangelos, Panagiotis Sarantis, Michalis V. Karamouzis, Philippe Vielh, and Stamatios Theocharis. "The Controversial Role of Autophagy in Ewing Sarcoma Pathogenesis—Current Treatment Options." Biomolecules 11, no. 3 (February 26, 2021): 355. http://dx.doi.org/10.3390/biom11030355.

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Ewing Sarcoma (ES) is a rare, aggressive, and highly metastasizing cancer in children and young adults. Most ES cases carry the fusion of the Ewing Sarcoma Breakpoint Region 1 (EWSR1) and FLI1 (Friend leukemia virus integration site 1) genes, leading to an EWS–FLI1 fused protein, which is associated with autophagy, a homeostatic and catabolic mechanism under normal and pathological conditions. Following such interesting and controversial data regarding autophagy in ES, many clinical trials using modulators of autophagy are now underway in this field. In the present review, we summarize current data and clinical trials that associate autophagy with ES. In vitro studies highlight the controversial role of autophagy as a tumor promoter or a tumor suppressor mechanism in ES. Clinical and in vitro studies on ES, together with the autophagy modulators, suggest that caution should be adopted in the application of autophagy as a therapeutic target. Monitoring and targeting autophagy in every ES patient could eliminate the need for targeting multiple pathways in order to achieve the maximum beneficial effect. Future studies are required to focus on which ES patients are affected by autophagy modulators in order to provide novel and more efficient therapeutic protocols for patients with ES based on the current autophagy status of the tumors.

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Thomsen, Christer, Sameer Udhane, Rikard Runnberg, Gerhard Wiche, Anders Ståhlberg, and Pierre Åman. "Fused in sarcoma (FUS) interacts with the cytolinker protein plectin: Implications for FUS subcellular localization and function." Experimental Cell Research 318, no. 5 (March 2012): 653–61. http://dx.doi.org/10.1016/j.yexcr.2011.12.019.

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Shimamura, Mai, Akane Kyotani, Yumiko Azuma, Hideki Yoshida, Thanh Binh Nguyen, Ikuko Mizuta, Tomokatsu Yoshida, et al. "Genetic link between Cabeza, a Drosophila homologue of Fused in Sarcoma (FUS), and the EGFR signaling pathway." Experimental Cell Research 326, no. 1 (August 2014): 36–45. http://dx.doi.org/10.1016/j.yexcr.2014.06.004.

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Yu, Xiaolong, Zhe Zhao, Hongrui Shen, Qi Bing, Nan Li, and Jing Hu. "Clinical and Genetic Features of Patients with Juvenile Amyotrophic Lateral Sclerosis with Fused in Sarcoma (FUS) Mutation." Medical Science Monitor 24 (December 3, 2018): 8750–57. http://dx.doi.org/10.12659/msm.913724.

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Baborie, A., E. Jaros, T. D. Griffiths, P. Momeni, R. Perry, and D. M. A. Mann. "Frontotemporal lobar degeneration in a very young patient is associated with fused in sarcoma (FUS) pathological changes." Neuropathology and Applied Neurobiology 38, no. 1 (January 6, 2012): 101–4. http://dx.doi.org/10.1111/j.1365-2990.2011.01209.x.

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Liu, Xuehui, Chunyan Niu, Jintao Ren, Jiayu Zhang, Xiaodong Xie, Haining Zhu, Wei Feng, and Weimin Gong. "The RRM domain of human fused in sarcoma protein reveals a non-canonical nucleic acid binding site." Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1832, no. 2 (February 2013): 375–85. http://dx.doi.org/10.1016/j.bbadis.2012.11.012.

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Bahia, Valéria Santoro, Leonel Tadao Takada, and Vincent Deramecourt. "Neuropathology of frontotemporal lobar degeneration: A review." Dementia & Neuropsychologia 7, no. 1 (March 2013): 19–26. http://dx.doi.org/10.1590/s1980-57642013dn70100004.

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ABSTRACT Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile dementia. Three main clinical variants are widely recognized within the FTLD spectrum: the behavioural variant of frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). FTLD represents a highly heterogeneous group of neurodegenerative disorders which are best classified according to the main protein component of pathological neuronal and glial inclusions. The most common pathological class of FTLD is associated with the TDP-43 protein (FTLD-TDP), while FTLD-Tau is considered slightly less common while the FTLD-FUS (Fused in sarcoma protein) pathology is rare. In this review, these three major pathological types of FTLD are discussed.

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Chornenka, Karina, Veronica Hirsch-Reinshagen, Mari Perez-Rosendahl, Howard Feldman, Freddi Segal-Gidan, Harry V. Vinters, and Ian R. Mackenzie. "Expanding the Phenotype of Frontotemporal Lobar Degeneration With FUS-Positive Pathology (FTLD-FUS)." Journal of Neuropathology & Experimental Neurology 79, no. 7 (June 1, 2020): 809–12. http://dx.doi.org/10.1093/jnen/nlaa045.

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Abstract Atypical frontotemporal lobar degeneration with ubiquitin-positive inclusions (aFTLD-U) is an uncommon cause of frontotemporal dementia characterized by fused in sarcoma-positive inclusions. It is classified as a subtype of frontotemporal lobar degeneration with FUS pathology. Cases with aFTLD-U pathology typically display an early onset of symptoms and severe psychobehavioral changes in the absence of significant aphasia, cognitive-intellectual dysfunction or motor features. This phenotype is regarded as being sufficiently unusual and consistent as to allow antemortem diagnosis with a high degree of accuracy. In this report, we describe 2 cases with aFTLD-U pathology that broaden the associated phenotype to include later age of onset, milder behavioral abnormalities and early memory and language impairment.

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Boulander, Emmanuelle, Renan Duprez, Eric Delabesse, Jean Gabarre, Elizabeth Macintyre, and Antoine Gessain. "Oligoclonal/Multiclonal Pattern of Kaposi Sarcoma-Associated Herpesvirus (KSHV/HHV-8) Episomes in Primary Effusion Lymphoma Cells." Blood 104, no. 11 (November 16, 2004): 1367. http://dx.doi.org/10.1182/blood.v104.11.1367.1367.

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Abstract Primary effusion lymphoma (PEL) is a rare non-Hodgkin lymphoma of B-cell lineage origin, developed in the serous body cavities and mostly observed in the course of HIV infection. PEL tumor cells are latently infected with Kaposi sarcoma-associated herpesvirus (KSHV) and in most cases, co-infected with Epstein-Barr virus (EBV). As a virological marker of clonality in PEL, we analyzed the fused terminal repeat regions (TR) of KSHV episomes using pulsed-field gel electrophoresis and Southern blot hybridization, in 15 primary PEL tumors including 10 EBV+ cases, and in 6 PEL cell lines. The cellular clonality was assessed on the same genomic DNA samples, by Southern blot and PCR detection of monoclonal immunoglobulin heavy chain (IgH) VDJ gene rearrangements, associated in the co-infected PEL with Southern blot analysis of the fused termini of EBV episomes. Monoclonal IgH gene rearrangements were detected in 87% (13/15) of primary PEL tumors using Southern blot, in 73% (11/15) using PCR analysis, and in all tumors considering both methods. All the co-infected PEL also displayed a monoclonal EBV infection. However, only 5 primary PEL tumors were found to be monoclonally infected with KSHV. In the 10 remaining cases, as well as in the KSHV+/EBV-negative BC-3 cell line, we found a biclonal (2 bands)(n=3) or an oligoclonal/multiclonal pattern (3–6 bands)(n=7) of KSHV episomes. KSHV infection of non-tumoral contaminating cells, superinfection mechanisms from lytically infected tumor cells, or viral integration events might explain such discordant findings between the viral and cellular clonality of PEL.

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Harrison, Alice Ford, and James Shorter. "RNA-binding proteins with prion-like domains in health and disease." Biochemical Journal 474, no. 8 (April 7, 2017): 1417–38. http://dx.doi.org/10.1042/bcj20160499.

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Approximately 70 human RNA-binding proteins (RBPs) contain a prion-like domain (PrLD). PrLDs are low-complexity domains that possess a similar amino acid composition to prion domains in yeast, which enable several proteins, including Sup35 and Rnq1, to form infectious conformers, termed prions. In humans, PrLDs contribute to RBP function and enable RBPs to undergo liquid–liquid phase transitions that underlie the biogenesis of various membraneless organelles. However, this activity appears to render RBPs prone to misfolding and aggregation connected to neurodegenerative disease. Indeed, numerous RBPs with PrLDs, including TDP-43 (transactivation response element DNA-binding protein 43), FUS (fused in sarcoma), TAF15 (TATA-binding protein-associated factor 15), EWSR1 (Ewing sarcoma breakpoint region 1), and heterogeneous nuclear ribonucleoproteins A1 and A2 (hnRNPA1 and hnRNPA2), have now been connected via pathology and genetics to the etiology of several neurodegenerative diseases, including amyotrophic lateral sclerosis, frontotemporal dementia, and multisystem proteinopathy. Here, we review the physiological and pathological roles of the most prominent RBPs with PrLDs. We also highlight the potential of protein disaggregases, including Hsp104, as a therapeutic strategy to combat the aberrant phase transitions of RBPs with PrLDs that likely underpin neurodegeneration.

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