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Mouse Anti-FRG1 (AA 1-259) Recombinant Antibody (CBXF-0901) (CBMAB-F0307-CQ)

This product is a mouse antibody that recognizes FRG1 (AA 1-259). The antibody CBXF-0901 can be used for immunoassay techniques such as: ELISA, IF, WB.
See all FRG1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXF-0901
Antibody Isotype
IgG2a
Application
ELISA, IF, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2a
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
Buffer
PBS, pH 7.2
Preservative
0.09% sodium azide
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
AA 1-259

Target

Full Name
FSHD Region Gene 1
Introduction
This gene maps to a location 100 kb centromeric of the repeat units on chromosome 4q35 which are deleted in facioscapulohumeral muscular dystrophy (FSHD). It is evolutionarily conserved and has related sequences on multiple human chromosomes but DNA sequence analysis did not reveal any homology to known genes. In vivo studies demonstrate the encoded protein is localized to the nucleolus.
Entrez Gene ID
2483
UniProt ID
Q14331
Alternative Names
FSHD Region Gene 1; FSHD Region Gene 1 Protein; Facioscapulohumeral Muscular Dystrophy Region Gene-1; Protein FRG1; FRG1A; FSG1;
Function
Binds to mRNA in a sequence-independent manner. May play a role in regulation of pre-mRNA splicing or in the assembly of rRNA into ribosomal subunits. May be involved in mRNA transport. May be involved in epigenetic regulation of muscle differentiation through regulation of activity of the histone-lysine N-methyltransferase KMT5B.
Biological Process
mRNA splicing, via spliceosome Source: UniProtKB
muscle organ development Source: UniProtKB-KW
rRNA processing Source: UniProtKB-KW
Cellular Location
Cytoplasm; Cajal body; Nucleolus; Z line. Localization changes during myogenesis from mainly cytoplasmic in undifferentiated myoblasts, to strongly nucleolar in early myotubes and back to cytoplasmic 5 days post-differentiation (PubMed:20970242). Localized at the Z-line in the sarcomere of matured myotubes 8 days post-differentiation (PubMed:20970242).
Involvement in disease
Facioscapulohumeral muscular dystrophy 1 (FSHD1):
The gene represented in this entry may be involved in disease pathogenesis. Overexpression of human FRG1 in mice leads to development of facioscapulohumeral muscular dystrophy (FSHD1)-like symptoms such as kyphosis, progressive muscle dystrophy and skeletal muscle atrophy (PubMed:16341202). It also causes aberrant pre-mRNA splicing of TNNT3 and MTMR1, affects the localization and activity of KMT5B, and leads to increased levels of EID3, resulting in inhibited muscle differentiation (PubMed:23720823). These results suggest that FSHD1 results from inappropriate overexpression of FRG1 which leads to abnormal alternative splicing of specific pre-mRNAs. A degenerative muscle disease characterized by slowly progressive weakness of the muscles of the face, upper-arm, and shoulder girdle. The onset of symptoms usually occurs in the first or second decade of life. Affected individuals usually present with impairment of upper extremity elevation. This tends to be followed by facial weakness, primarily involving the orbicularis oris and orbicularis oculi muscles.

Palo, A., Patel, S. A., Sahoo, B., Chowdary, T. K., & Dixit, M. (2023). FRG1 is a direct transcriptional regulator of nonsense-mediated mRNA decay genes. Genomics, 115(1), 110539.

Mukherjee, B., Brahma, P., Mohapatra, T., Chawla, S., & Dixit, M. (2023). Reduced FRG1 expression promotes angiogenesis via activation of the FGF2‐mediated ERK/AKT pathway. FEBS Open Bio.

Mukherjee, B., Tiwari, A., Palo, A., Pattnaik, N., Samantara, S., & Dixit, M. (2022). Reduced expression of FRG1 facilitates breast cancer progression via GM-CSF/MEK-ERK axis by abating FRG1 mediated transcriptional repression of GM-CSF. Cell Death Discovery, 8(1), 442.

Khan, R., Palo, A., & Dixit, M. (2021). Role of FRG1 in predicting the overall survivability in cancers using multivariate based optimal model. Scientific Reports, 11(1), 22505.

Mukherjee, B., Tiwari, A., & Dixit, M. (2020). Novel tumor suppressor FRG1 reduces cancerous properties of breast cancer cell lines by regulating ERK pathway, irrespective of molecular subtypes. Cancer Research, 80(16_Supplement), 5969-5969.

Tiwari, A., Mukherjee, B., Hassan, M. K., Pattanaik, N., Jaiswal, A. M., & Dixit, M. (2019). Reduced FRG1 expression promotes prostate cancer progression and affects prostate cancer cell migration and invasion. BMc cancer, 19, 1-15.

Mah, J. K., & Chen, Y. W. (2018). A pediatric review of facioscapulohumeral muscular dystrophy. Journal of Pediatric Neurology, 16(04), 222-231.

Himeda, C. L., Jones, T. I., Virbasius, C. M., Zhu, L. J., Green, M. R., & Jones, P. L. (2018). Identification of epigenetic regulators of DUX4-fl for targeted therapy of facioscapulohumeral muscular dystrophy. Molecular Therapy, 26(7), 1797-1807.

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

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