SMN-Exon7 Antibody 1 Publications

Mouse Monoclonal| Catalog number: 60255-1-Ig |CloneNo.: 3A8G11

  • Print page
  • Download PDF

Be the first to review this product


-_-

Freight/Packing

Con: 150 μg/150 μl

Choose size:

Please visit your regions distributor:


Species specificity:
human, mouse

Positive WB detected in:
HEK-293 cells, HeLa cells, HepG2 cells

Positive IF detected in:
HepG2 cells

Recommended dilution:
WB : 1:500-1:2000
IF : 1:50-1:500

Product Information


Source:
Mouse

Purification method:
Protein G purification

Isotype:
IgG1

Storage:
PBS with 0.02% sodium azide and 50% glycerol pH 7.3. Store at -20oC. Aliquoting is unnecessary for -20oC storage.

Immunogen Information


Full name:
survival of motor neuron 1, telomeric

Calculated molecular weight:
294aa,32 kDa

Observed molecular weight:
40 kDa

GenBank accession number:

Gene ID (NCBI):

Gene symbol
SMN1

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

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-ternimal region (275-294aa) encoded by the exon 7.


Back
to top