SMN-Exon7 Monoclonal antibody, PBS Only

SMN-Exon7 Monoclonal Antibody for WB, IF, Indirect ELISA

Host / Isotype

Mouse / IgG1

Reactivity

human

Applications

WB, IF, Indirect ELISA

Conjugate

Unconjugated

CloneNo.

3A8G11

Cat no : 60255-1-PBS

Synonyms

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



Tested Applications

Recommended dilution

ApplicationDilution
It is recommended that this reagent should be titrated in each testing system to obtain optimal results.

Product Information

60255-1-PBS targets SMN-Exon7 in WB, IF, Indirect ELISA applications and shows reactivity with human samples.

Tested Reactivity human
Host / Isotype Mouse / IgG1
Class Monoclonal
Type Antibody
Immunogen SMN-Exon7 fusion protein Ag16615 相同性解析による交差性が予測される生物種
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
Conjugate Unconjugated
Form Liquid
Purification MethodProtein A purification
Storage Buffer PBS Only
Storage ConditionsStore at -80°C.

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.