CoraLite®594-conjugated SMN-Exon7 Monoclonal antibody

SMN-Exon7 Monoclonal Antibody for IF

Host / Isotype

Mouse / IgG1

Reactivity

human, mouse

Applications

IF/ICC

Conjugate

CoraLite®594 Fluorescent Dye

CloneNo.

3A8G11

Cat no : CL594-60255

Synonyms

Component of gems 1, Gemin 1, SMA, SMA@, SMA1, SMA2, SMA3, SMA4, SMN, SMN1, SMN2, SMNC, SMN-Exon7, SMNT, Survival motor neuron protein, T BCD541

---

Validation Data Gallery

• 

  IF Staining of HepG2 using CL594-60255

  Immunofluorescent analysis of (-20°C Ethanol) fixed HepG2 cells using CoraLite®594 SMN-Exon7 antibody (CL594-60255, Clone: 3A8G11 ) at dilution of 1:200.

---

Tested Applications

• Positive IF/ICC detected in: HepG2 cells

Recommended dilution

• Immunofluorescence (IF)/ICC: IF/ICC : 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.

Product Information

CL594-60255 targets SMN-Exon7 in IF/ICC applications and shows reactivity with human, mouse samples.

• Tested Reactivity: human, mouse
• 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 Number: BC062723
• Gene Symbol: SMN
• Gene ID (NCBI): 6606
• RRID: AB_2883449
• Conjugate: CoraLite®594 Fluorescent Dye
• Excitation/Emission Maxima Wavelengths: 588 nm / 604 nm
• Form: Liquid
• Purification Method: Protein G purification
• Storage Buffer: PBS with 50% Glycerol, 0.05% Proclin300, 0.5% BSA, pH 7.3.
• Storage Conditions: Store at -20°C. Avoid exposure to light. Stable for one year after shipment. Aliquoting is unnecessary for -20^oC storage.

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.