Mouse Anti-FRG1 Recombinant Antibody (4A5) (CBMAB-A3171-LY)

Name: Mouse Anti-FRG1 Recombinant Antibody (4A5)
SKU: CBMAB-A3171-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

4A5

Application

WB, ELISA

Immunogen

FRG1 (AAH53997, 1 a.a. ~ 258 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Specificity

Human

Antibody Isotype

IgG2a, κ

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

Purity

> 95% Purity determined by SDS-PAGE.

Storage

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

More Infomation

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. [provided by RefSeq]

Entrez Gene ID

2483

UniProt ID

Q14331

Alternative Names

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Antibody Pairs

FRG1 Matched Antibody Pair (452) (CAT#: APMAB-452LY)

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FRG1 Matched Antibody Pair (452) (CAT#: APMAB-452LY)

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Isotype control

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

Custom Antibody Labeling

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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