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Rabbit Anti-FXR1 Recombinant Antibody (CBXF-2085) (CBMAB-F2023-CQ)


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Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat, Monkey
Clone
CBXF-2085
Antibody Isotype
IgG
Application
WB, IF

Basic Information

Specificity
Human, Mouse, Rat, Monkey
Antibody Isotype
IgG
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!]

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

Target

Full Name
FMR1 Autosomal Homolog 1
Introduction
The protein encoded by this gene is an RNA binding protein that interacts with the functionally-similar proteins FMR1 and FXR2. These proteins shuttle between the nucleus and cytoplasm and associate with polyribosomes, predominantly with the 60S ribosomal subunit. Three transcript variants encoding different isoforms have been found for this gene.
Entrez Gene ID
Human8087
Mouse14359
Rat361927
Monkey710170
UniProt ID
HumanP51114
MouseQ61584
RatQ5XI81
MonkeyF6W8C6
Alternative Names
FMR1 Autosomal Homolog 1; Fragile X Mental Retardation, Autosomal Homolog 1; HFXR1p; Fragile X Mental Retardation Autosomal Homolog Variant P5FK; Fragile X Mental Retardation Autosomal Homolog Variant P1K; Fragile X Mental Retardation Autosomal Homolog Variant P2K;
Function
RNA-binding protein required for embryonic and postnatal development of muscle tissue (PubMed:30770808).

May regulate intracellular transport and local translation of certain mRNAs (By similarity).
Biological Process
Apoptotic process Source: ProtInc
Cell differentiation Source: UniProtKB-KW
Muscle organ development Source: UniProtKB
Negative regulation of translation Source: GO_Central
Positive regulation of gene silencing by miRNA Source: UniProtKB
Positive regulation of protein phosphorylation Source: GO_Central
Positive regulation of response to DNA damage stimulus Source: GO_Central
Positive regulation of translation Source: GO_Central
Regulation of alternative mRNA splicing, via spliceosome Source: GO_Central
Regulation of filopodium assembly Source: GO_Central
Regulation of mRNA stability Source: GO_Central
Skeletal muscle organ development Source: UniProtKB
Cellular Location
Cytosol. Adjacent to Z-lines in muscles.
Involvement in disease
Myopathy, congenital, with respiratory insufficiency and bone fractures (MYORIBF):
An autosomal recessive muscular disorder characterized by severe hypotonia apparent at birth, poor feeding, ulnar deviation of the hands, laterally deviated feet, fractures of the long bones, respiratory insufficiency due to muscle weakness, and death in infancy.
Myopathy, congenital proximal, with minicore lesions (MYOPMIL):
An autosomal recessive, slowly progressive muscular disorder characterized by primarily proximal muscle weakness, neonatal hypotonia leading to delayed motor development, mildly delayed walking in childhood, and difficulty running or climbing. Cardiac function is unaffected, but most patients have obstructive sleep apnea. Muscle biopsy shows type 1 fiber predominance with disorganized Z-lines and minicores that disrupt the myofibrillar striation pattern.
PTM
Arg-445 is dimethylated, probably to asymmetric dimethylarginine.
More Infomation

Shimizu, H., & Hohjoh, H. (2023). FMRP, FXR1 protein and Dlg4 mRNA, which are associated with fragile X syndrome, are involved in the ubiquitin–proteasome system. Scientific Reports, 13(1), 1956.

Herring, J., Johnson, K., & Richstein, J. (2022). The use of “retardation” in FRAXA, FMRP, FMR1 and other designations. Cells, 11(6), 1044.

Wang, B., Yin, H., Zhang, H., & Wang, T. (2021). circNRIP1 facilitates keloid progression via FXR1‑mediated upregulation of miR‑503‑3p and miR‑503‑5p. International Journal of Molecular Medicine, 47(5), 1-13.

Smith, J. A., Curry, E. G., Blue, R. E., Roden, C., Dundon, S. E., Rodríguez-Vargas, A., ... & Giudice, J. (2020). FXR1 splicing is important for muscle development and biomolecular condensates in muscle cells. Journal of Cell Biology, 219(4).

Khlghatyan, J., Evstratova, A., Bozoyan, L., Chamberland, S., Chatterjee, D., Marakhovskaia, A., ... & Beaulieu, J. M. (2020). Fxr1 regulates sleep and synaptic homeostasis. The EMBO Journal, 39(21), e103864.

Cao, H., Gao, R., Yu, C., Chen, L., & Feng, Y. (2019). The RNA-binding protein FXR1 modulates prostate cancer progression by regulating FBXO4. Functional & integrative genomics, 19, 487-496.

Smith, J. A., Curry, E. G., Blue, R. E., Roden, C., Dundon, S. E., Rodríguez-Vargas, A., ... & Giudice, J. (2019). Regulation of FXR1 by alternative splicing is required for muscle development and controls liquid-like condensates in muscle cells. bioRxiv, 818476.

Nordio, L., Marques, A. T., Lecchi, C., Luciano, A. M., Stefanello, D., & Giudice, C. (2018). Immunohistochemical expression of FXR1 in canine normal tissues and melanomas. Journal of Histochemistry & Cytochemistry, 66(8), 585-593.

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

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We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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