Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death

The gene FUS (also known as TLS (for translocated in liposarcoma) and hnRNP P2) is translocated with the gene encoding the transcription factor ERG-1 in human myeloid leukaemias. Although the functions of wild-type FUS are unknown, the protein contains an RNA-recognition motif and is a component of...

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Bibliographic Details
Published in:	Nature genetics Vol. 24; no. 2; pp. 175 - 179
Main Authors:	Hicks, Geoffrey G, Singh, Nagendra, Nashabi, Abudi, Mai, Sabine, Bozek, Gracjan, Klewes, Ludger, Arapovic, Djula, White, Erica K, Koury, Mark J, Oltz, Eugene M, Van Kaer, Luc, Ruley, H. E
Format:	Journal Article
Language:	English
Published:	London Nature Publishing Group 01-02-2000
Subjects:	Animals Animals, Newborn B cells B-Lymphocytes - immunology Biological and medical sciences Bone Marrow Cells - immunology Cell differentiation, maturation, development, hematopoiesis Cell physiology Chimera Cloning Crosses, Genetic Deoxyribonucleic acid DNA ERG-1 protein Female Fundamental and applied biological sciences. Psychology FUS gene Fus protein Gene mutations Genotype Health aspects Heterogeneous-Nuclear Ribonucleoproteins hnRNA Humans Identification and classification Liver - immunology Lymphocyte Activation Lymphocytes Male Mice Mice, Inbred C57BL Mice, Knockout Molecular and cellular biology Mutation Oncogenes Physiological aspects Proteins Restriction Mapping Ribonucleoproteins - deficiency Ribonucleoproteins - genetics Ribonucleoproteins - metabolism RNA-Binding Protein FUS RNA-Binding Proteins - metabolism Spleen - immunology Transcription factors Canada United States > US Tennessee Nashville Tennessee Vertebrata Phenotype Mammalia Cell function Stability Mouse Rodentia DNA binding protein Development B-Lymphocyte Mutation Genome
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Summary:	The gene FUS (also known as TLS (for translocated in liposarcoma) and hnRNP P2) is translocated with the gene encoding the transcription factor ERG-1 in human myeloid leukaemias. Although the functions of wild-type FUS are unknown, the protein contains an RNA-recognition motif and is a component of nuclear riboprotein complexes. FUS resembles a transcription factor in that it binds DNA, contributes a transcriptional activation domain to the FUS-ERG oncoprotein and interacts with several transcription factors in vitro. To better understand FUS function in vivo, we examined the consequences of disrupting Fus in mice. Our results indicate that Fus is essential for viability of neonatal animals, influences lymphocyte development in a non-cell-intrinsic manner, has an intrinsic role in the proliferative responses of B cells to specific mitogenic stimuli and is required for the maintenance of genomic stability. The involvement of a nuclear riboprotein in these processes in vivo indicates that Fus is important in genome maintenance.
Bibliography:	ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-1 content type line 23 ObjectType-Article-1 ObjectType-Feature-2
ISSN:	1061-4036 1546-1718
DOI:	10.1038/72842