Cell Signaling Smn1 (2f1) Mouse mAb

Name: Cell Signaling Smn1 (2f1) Mouse mAb
SKU: CSSPQ-12976S
Price: 348.00 USD

List Price $348.00 Your Price $348.00

Cell Signaling Smn1 (2f1) Mouse mAb - CSSPQ (Additional S&H or Hazmat Fees May Apply)

NETA PART: CSSPQ-12976S

MFG.PART: 12976S

UNSPSC: 12352203

Manufacturer: Cell Signaling

Size	100 µl
Reactivity	H Mk
Sensitivity	Endogenous
Molecular Weight (kDa)	35
Source/Isotype	Mouse IgG1
Application/Dilution	{Western Blotting: 1:1000, Immunoprecipitation: 1:50, Immunofluorescence (Immunocytochemistry): 1:400}
Storage	Supplied in 10 mm sodium HEPES (pH 7.5), 150 mm NaCl, 100 µg/mL BSA, 50% glycerol and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.
Specificity/Sensitivity	SMN1 (2F1) Mouse mAb recognizes endogenous levels of total SMN1 protein.
Species Reactivity	Human, Monkey
Source/Purification	Monoclonal antibody is produced by immunizing animals with recombinant protein specific to the carboxy terminus of human SMN1 protein.
Background	Survival of Motor Neuron 1 (SMN1) is essential for the maturation of small nuclear ribonucleoproteins (snRNPs) (1,2). SMN1 plays a role in the assembly of spliceosomal snRNPs in the cytoplasm, together with the Gemin proteins, and may also participate in the transport of snRNPs into the nucleus (3-6). SMN1 also participates in the maturation and turnover of snRNPs in nuclear foci Gemini bodies (gems) (7). In addition to the maturation of spliceosomal snRNPs, SMN1 has also been proposed to directly regulate pre-mRNA splicing (8). Researchers have found mutations and deletions of the SMN1 gene are found in 95% of Spinal Muscular Atrophy (SMA) neuromuscular disorder cases (1,9).