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SMN-Exon7 Monoclonal antibody

SMN-Exon7 Monoclonal Antibody for IF, WB,ELISA

Host / Isotype

Mouse / IgG1

Reactivity

human

Applications

WB, IF, ELISA

Conjugate

Unconjugated

CloneNo.

3A8G11

Cat no : 60255-1-Ig

Synonyms

Component of gems 1, Gemin 1, SMN, SMN1, SMN2, SMNC, SMN-Exon7, SMNT, Survival motor neuron protein, T BCD541

Validation Data Gallery

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  • Western Blot (WB) analysis of HEK-293 cells using SMN-Exon7 Monoclonal antibody (60255-1-Ig)

    WB analysis of HEK-293 using 60255-1-Ig

    HEK-293 cells were subjected to SDS PAGE followed by western blot with 60255-1-Ig (SMN-Exon7 antibody) at dilution of 1:1000 incubated at room temperature for 1.5 hours.

  • Western Blot (WB) analysis of HepG2 cells using SMN-Exon7 Monoclonal antibody (60255-1-Ig)

    WB analysis of HepG2 using 60255-1-Ig

    HepG2 cells were subjected to SDS PAGE followed by western blot with 60255-1-Ig (SMN-Exon7 antibody at dilution of 1:1000 incubated at room temperature for 1.5 hours.

  • Western Blot (WB) analysis of HeLa cells using SMN-Exon7 Monoclonal antibody (60255-1-Ig)

    WB analysis of HeLa using 60255-1-Ig

    HeLa cells were subjected to SDS PAGE followed by western blot with 60255-1-Ig (SMN-Exon7 antibody at dilution of 1:1000 incubated at room temperature for 1.5 hours.

  • Immunofluorescence (IF) / fluorescent staining of HepG2 cells using SMN-Exon7 Monoclonal antibody (60255-1-Ig)

    IF Staining of HepG2 using 60255-1-Ig

    Immunofluorescent analysis of HepG2 cells using 60255-1-Ig (SMN-Exon7 antibody) at dilution of 1:50 and Alexa Fluor 488-conjugated AffiniPure Goat Anti-Mouse IgG (H+L).

Tested Applications

Positive WB detected inHEK-293 cells, HeLa cells, HepG2 cells
Positive IF detected inHepG2 cells

Recommended dilution

ApplicationDilution
Western Blot (WB)WB : 1:500-1:2000
Immunofluorescence (IF)IF : 1:50-1:500
It is recommended that this reagent should be titrated in each testing system to obtain optimal results.
Sample-dependent, Check data in validation data gallery.

Published Applications

WBSee 1 publications below

Product Information

The immunogen of 60255-1-Ig is SMN-Exon7 Fusion Protein expressed in E. coli.

Tested Reactivity human
Host / Isotype Mouse / IgG1
Class Monoclonal
Type Antibody
Immunogen SMN-Exon7 fusion protein Ag16615
Full Name survival of motor neuron 1, telomeric
Calculated Molecular Weight 294 aa, 32 kDa
Observed Molecular Weight 40 kDa
GenBank Accession NumberBC062723
Gene Symbol SMN1
Gene ID (NCBI) 6606
RRIDAB_2881376
Conjugate Unconjugated
Form Liquid
Purification MethodProtein A purification
Storage Buffer PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
Storage ConditionsStore at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. 20ul sizes contain 0.1% BSA.

Background Information

Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of anterior horn cells in the spinal cord and concomitant symmetrical muscle weakness and atrophy (PMID: 16364894 ). SMA is caused by deletion or mutations of the survival motor neuron (SMN1) gene. SMA patients lack a functional SMN1 gene, but they possess an intact SMN2 gene, which though nearly identical to SMN1, is only partially functional (PMID: 17355180). A large majority of SMN2 transcripts lack exon 7, resulting in production of a truncated, less stable SMN protein (PMID: 10369862). The level of SMN protein correlates with phenotypic severity of SMA. This antibody, 60255-1-Ig, raised against the C-terminal region (275-294aa) encoded by the exon 7.

Protocols

Product Specific Protocols
WB protocol for SMN-Exon7 antibody 60255-1-IgDownload protocol
IF protocol for SMN-Exon7 antibody 60255-1-IgDownload protocol
Standard Protocols
Click here to view our Standard Protocols

Publications

SpeciesApplicationTitle
WB

Science

Base editing rescue of spinal muscular atrophy in cells and in mice

Authors - Mandana Arbab
View Article