This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.
product summary
company name :
Enzo Life Sciences
other brands :
Stressgen, BioMol, Alexis Corporation, Assay Designs, Alexis
product type :
antibody
product name :
SMN1 monoclonal antibody (2B1)
catalog :
ADI-NBA-202-050
quantity :
50 µg
clonality :
monoclonal
host :
mouse
conjugate :
nonconjugated
clone name :
2B1

The same clone is also sold as:
reactivity :
human
application :
western blot
citations: 1
Reference
Zaworski P, von Herrmann K, Taylor S, Sunshine S, McCarthy K, Risher N, et al. SMN Protein Can Be Reliably Measured in Whole Blood with an Electrochemiluminescence (ECL) Immunoassay: Implications for Clinical Trials. PLoS ONE. 2016;11:e0150640 pubmed publisher
product information
Product ID :
ADI-NBA-202
Catalog Number :
ADI-NBA-202-050
Product Name :
SMN1 monoclonal antibody (2B1)
Host species :
Mouse
Clonality :
Monoclonal
Product Type :
Antibody
Size :
50 µg
Clone :
2B1
Isotype :
IgG
SPECIES REACTIVITY :
Human
Background :
Survival Motor Neuron (SMN) is a ~38 kDa protein produced chiefly by the SMN1 gene, located on the telomeric portion of chromosome 5q. A nearly identical centromeric copy of the gene (SMN2) also produces a small amount of full-length SMN protein, but due to a translationally silent C(R)T transition that results in alternative splicing of the pre-mRNA, most of the resulting SMN is truncated, causing reduced protein stability and lower overall SMN levels. Deletion or mutation of the SMN1 gene results in a reduced level of full-length SMN protein and manifests as a range of neuromuscular phenotypes in humans as the disease spinal muscular atrophy (SMA). SMA is characterized by muscle weakness and atrophy, functional disability and is the most common lethal genetic disease of infants and toddlers. Approximately one in 35 adults is a carrier of the SMN1 mutation. The incidence of SMA is 1 in 6,000 to 1 in 10,000 live births. SMN protein is present in the cell cytoplasm, and also in the nucleus where it is concentrated in “gem” structures associated with Cajal bodies. SMN protein is a constituent of Gemin-containing complexes, and is thought to participate in many aspects of RNA metabolism. SMN complexes have been shown to mediate the assembly of uridine-rich small nuclear ribonucleoproteins (snRNPs), which in turn act as critical components of spliceosomes.
ALTnames :
Survival Motor Neuron
Immunogen :
Recombinant fragment corresponding to the carboxy-terminus of human SMN1.
Buffer :
Liquid. In PBS, pH 7.2, containing 0.09% sodium azide.
Uses :
Detects a band of ~38kDa by Western blot.
Application Summary :
WB
Storage :
-20°C
company information
Enzo Life Sciences
5777 Hines Drive
Ann Arbor, MI 48108
info-us@enzolifesciences.
https://www.enzolifesciences.com
1-800-942-0430
headquarters: USA
Enzo Life Sciences, incorporating ALEXIS® Biochemicals and BIOMOL® International, offers a comprehensive panel of innovative life science reagents. Antibodies, enzymes, proteins, small molecule inhibitors, product libraries, and assay kits are available for research in Cancer, Cell Death, Epigenetics, Genomics, Immunology, Neuroscience, Signal Transduction, and Ubiquitin & Proteasomes, Enabling Discovery in Life Science(tm). Visit www.enzolifesciences.com.