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Mouse Anti-FUS Recombinant Antibody (246) (CBMAB-AP2407LY)


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Summary

Host Animal
Mouse
Specificity
Human, Mouse
Clone
246
Antibody Isotype
IgG1
Application
ELISA, IF, IHC, WB

Basic Information

Immunogen
Fusion protein of FUS/TLS
Specificity
Human, Mouse
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
FUS RNA Binding Protein
Introduction
This gene encodes a multifunctional protein component of the heterogeneous nuclear ribonucleoprotein (hnRNP) complex. The hnRNP complex is involved in pre-mRNA splicing and the export of fully processed mRNA to the cytoplasm. This protein belongs to the FET family of RNA-binding proteins which have been implicated in cellular processes that include regulation of gene expression, maintenance of genomic integrity and mRNA/microRNA processing. Alternative splicing results in multiple transcript variants. Defects in this gene result in amyotrophic lateral sclerosis type 6. [provided by RefSeq, Sep 2009]
Entrez Gene ID
Human2521
Mouse233908
UniProt ID
HumanP35637
MouseP56959
Alternative Names
FUS RNA Binding Protein; Heterogeneous Nuclear Ribonucleoprotein P2; Translocated In Liposarcoma Protein; 75 KDa DNA-Pairing Protein; Fused In Sarcoma; Oncogene FUS; Oncogene TLS; POMP75; TLS; Fusion (Involved In T(12; 16) In Malignant Liposarcoma); Fusion, Derived From T(12; 16) Malignant Liposarcoma;
Function
DNA/RNA-binding protein that plays a role in various cellular processes such as transcription regulation, RNA splicing, RNA transport, DNA repair and damage response (PubMed:27731383).

Binds to nascent pre-mRNAs and acts as a molecular mediator between RNA polymerase II and U1 small nuclear ribonucleoprotein thereby coupling transcription and splicing (PubMed:26124092).

Binds also its own pre-mRNA and autoregulates its expression; this autoregulation mechanism is mediated by non-sense-mediated decay (PubMed:24204307).

Plays a role in DNA repair mechanisms by promoting D-loop formation and homologous recombination during DNA double-strand break repair (PubMed:10567410).

In neuronal cells, plays crucial roles in dendritic spine formation and stability, RNA transport, mRNA stability and synaptic homeostasis (By similarity).
Biological Process
Cellular response to calcium ion Source: Ensembl
mRNA stabilization Source: MGI
Positive regulation of double-strand break repair via homologous recombination Source: UniProtKB
Protein homooligomerization Source: UniProtKB
Regulation of RNA splicing Source: UniProtKB
Regulation of transcription, DNA-templated Source: UniProtKB
Regulation of transcription by RNA polymerase II Source: UniProtKB
RNA splicing Source: UniProtKB
Cellular Location
Nucleus. Displays a punctate pattern inside the nucleus and is excluded from nucleoli.
Involvement in disease
A chromosomal aberration involving FUS is found in a patient with malignant myxoid liposarcoma. Translocation t(12;16)(q13;p11) with DDIT3.
A chromosomal aberration involving FUS is a cause of acute myeloid leukemia (AML). Translocation t(16;21)(p11;q22) with ERG.
Angiomatoid fibrous histiocytoma (AFH):
The gene represented in this entry is involved in disease pathogenesis. A chromosomal aberration involving FUS is found in a patient with angiomatoid fibrous histiocytoma. Translocation t(12;16)(q13;p11.2) with ATF1 generates a chimeric FUS/ATF1 protein. A distinct variant of malignant fibrous histiocytoma that typically occurs in children and adolescents and is manifest by nodular subcutaneous growth. Characteristic microscopic features include lobulated sheets of histiocyte-like cells intimately associated with areas of hemorrhage and cystic pseudovascular spaces, as well as a striking cuffing of inflammatory cells, mimicking a lymph node metastasis.
Amyotrophic lateral sclerosis 6, with or without frontotemporal dementia (ALS6):
A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.
Tremor, hereditary essential 4 (ETM4):
A common movement disorder mainly characterized by postural tremor of the arms. Head, legs, trunk, voice, jaw, and facial muscles also may be involved. The condition can be aggravated by emotions, hunger, fatigue and temperature extremes, and may cause a functional disability or even incapacitation. Inheritance is autosomal dominant.
PTM
Arg-216 and Arg-218 are dimethylated, probably to asymmetric dimethylarginine.
Phosphorylated in its N-terminal serine residues upon induced DNA damage. ATM and DNA-PK are able to phosphorylate FUS N-terminal region.
More Infomation

Gebauer, F., Schwarzl, T., Valcárcel, J., & Hentze, M. W. (2021). RNA-binding proteins in human genetic disease. Nature Reviews Genetics, 22(3), 185-198.

Van Nostrand, E. L., Pratt, G. A., Yee, B. A., Wheeler, E. C., Blue, S. M., Mueller, J., ... & Yeo, G. W. (2020). Principles of RNA processing from analysis of enhanced CLIP maps for 150 RNA binding proteins. Genome biology, 21(1), 1-26.

Thelen, M. P., & Kye, M. J. (2020). The role of RNA binding proteins for local mRNA translation: implications in neurological disorders. Frontiers in Molecular Biosciences, 6, 161.

Van Nostrand, E. L., Freese, P., Pratt, G. A., Wang, X., Wei, X., Xiao, R., ... & Yeo, G. W. (2020). A large-scale binding and functional map of human RNA-binding proteins. Nature, 583(7818), 711-719.

Xiao, R., Chen, J. Y., Liang, Z., Luo, D., Chen, G., Lu, Z. J., ... & Fu, X. D. (2019). Pervasive chromatin-RNA binding protein interactions enable RNA-based regulation of transcription. Cell, 178(1), 107-121.

Loughlin, F. E., Lukavsky, P. J., Kazeeva, T., Reber, S., Hock, E. M., Colombo, M., ... & Allain, F. H. T. (2019). The solution structure of FUS bound to RNA reveals a bipartite mode of RNA recognition with both sequence and shape specificity. Molecular cell, 73(3), 490-504.

Tyzack, G. E., Luisier, R., Taha, D. M., Neeves, J., Modic, M., Mitchell, J. S., ... & Patani, R. (2019). Widespread FUS mislocalization is a molecular hallmark of amyotrophic lateral sclerosis. Brain, 142(9), 2572-2580.

Guo, L., Kim, H. J., Wang, H., Monaghan, J., Freyermuth, F., Sung, J. C., ... & Shorter, J. (2018). Nuclear-import receptors reverse aberrant phase transitions of RNA-binding proteins with prion-like domains. Cell, 173(3), 677-692.

St George-Hyslop, P., Lin, J. Q., Miyashita, A., Phillips, E. C., Qamar, S., Randle, S. J., & Wang, G. (2018). The physiological and pathological biophysics of phase separation and gelation of RNA binding proteins in amyotrophic lateral sclerosis and fronto-temporal lobar degeneration. Brain research, 1693, 11-23.

Maharana, S., Wang, J., Papadopoulos, D. K., Richter, D., Pozniakovsky, A., Poser, I., ... & Alberti, S. (2018). RNA buffers the phase separation behavior of prion-like RNA binding proteins. Science, 360(6391), 918-921.

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For research use only. Not intended for any clinical use.

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