Survival of Motor Neuron 1, Telomeric (Survival of Motor Neuron 1 (Telomeric), SMNT, Survival of Motor Neuron 1, Survival Motor Neuron 1 Protein, SMN1, BCD541, Component of Gems 1, Gemin 1, Gemin-1, SMA, SMA@, SMA1, SMA2, SMA3, SMA4, SMNC, Survival Motor

Survival of Motor Neuron 1 (SMN, Germin 1) is the central component of a large oligomeric complex (SMN complex), including Gemins 2-7, that is necessary and sufficient for the in vivo assembly of Sm proteins onto the small nuclear (sn)RNAs that mediate pre-mRNA splicing. The SMN complex is found in the cytoplasm and nucleus. Within the nucleus, this protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). Spinal muscular atrophy, a motor neuron disorder, results from reduced levels or a mutation in the Gemin 1 protein.\n\nApplications: \nSuitable for use in Western Blot, Immunoprecipitation and Immunocytochemistry. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1-2ug/ml\nOptimal dilutions to be determined by the researcher.\n\nPositive Control: \nA431 whole cell lysate\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

S8495-03

Size

50ul

Applications

ICC, IP, WB

Hosts

Mouse

Reactivities

Hum, Mouse, Xen/Amph

Form

Supplied as a liquid in PBS, pH 7.4, 15mM sodium azide.

P Type

Mab

Purity

Purified

Isotype

IgG1

References

1. Liu, Q., et al., EMBO J. 15: 3555-3565 (1996). 2. Charroux, B., et al., J. Cell Biol. 147: 1181-1193 (1999). 3. Paushkin, S., et al., Curr. Opin. Cell Biol. 14: 305-312 (2002). 4. Pellizzoni, L., et al., Proc. Natl. Acad. Sci. USA 96: 11,167-11,172 (1999). 5. Baccon, J., et al., J. Biol. Chem. 277: 31,957-31,962 (2002). 6. Pellizzoni, L., Science 298: 1775-1779 (2002).

Additional Info

Recognizes human Survival of Motor Neuron 1, Telomeric at ~35kD. Species Crossreactivity: Mouse, Xenopus laevis