Mouse Anti-FRG1 Recombinant Antibody (CBXF-0901) (V2LY-1225-LY88)

Name: Mouse Anti-FRG1 Recombinant Antibody (CBXF-0901)
SKU: V2LY-1225-LY88
Rating: 5 (1 reviews)

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Published Data

Mouse Anti-FRG1 Recombinant Antibody (CBXF-0901) (V2LY-1225-LY88) in WB

Alternative splicing changes are a primary consequence of FRG1 overexpression.

(a) Examples of alternative exons (Atl2) or introns (Ttn) spliced normally in the mouse model of Duchenne muscular dystrophy, mdx mice, and example of an alternative exon similarly altered in FRG1 and mdx mice (Ktn1). (b) RT-PCR analysis of mRNA splicing variants from proliferating (MB) and differentiating (MT) C2C12 muscle cells over-expressing FRG1. Examples of alternative splicing changes present in both MB and MT (Capn3), only in MB (Ablim1), or only in MT (Nasp). Numbers are the percentage of exon inclusion. Black boxes illustrate constitutive exons, white boxes alternatively spliced exons and double lines represent the affected intron. ...View More

Pistoni, M., Shiue, L., Cline, M. S., Bortolanza, S., Neguembor, M. V., Xynos, A., ... & Gabellini, D. (2013). Rbfox1 downregulation and altered calpain 3 splicing by FRG1 in a mouse model of Facioscapulohumeral muscular dystrophy (FSHD). PLoS genetics, 9(1), e1003186.

Datasheet

SDS

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

Basic Information

Host Animal

Mouse

Clone

CBXF-0901

Application

WB, IP, IF, ELISA

Immunogen

Recombinant human FRG1.

Host Species

Mouse

Specificity

Human, Mouse, Rat

Antibody Isotype

IgG2a, κ

Clonality

Monoclonal

Application Notes

Application	Note
ELISA	1:100-1:1,000
WB	1:100-1:1,000
IP	1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)	1:50-1:500

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

Format

Liquid

Buffer

PBS, gelatin

Preservative

Sodium azide

Concentration

0.1 mg/ml

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 freeze/thaw cycles.

More Infomation

Target

Full Name

FSHD Region Gene 1

Entrez Gene ID

Human	2483
Mouse	14300
Rat	686103

UniProt ID

Human	Q14331
Mouse	P97376
Rat	D3ZZK8

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)
Proteogenomic
Other Protocols

Alternative Versions

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

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

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