antibody

SMN1/SMN2 (Survival Motor Neuron Protein, Component of Gems 1, Gemin-1, SMN, SMNT, SMNC)

S8495-10B

100 ug

polyclonal

domestic goat

nonconjugated

SMN1/SMN2 (Survival Motor Neuron Protein, Component of Gems 1, Gemin-1, SMN, SMNT, SMNC)

goat

Pab

Antibodies

100 ug

IgG

SMN1/SMN2 is part of a 500kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. This gene may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7 which is thought to be an exon splice enhancer. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The full length protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Applications: Suitable for use in ELISA and Western Blot. Other applications not tested. Recommended Dilution: ELISA: 1:32,000 Western Blot: 0.3-1ug/ml, observed in human brain, cerebellum lysates on ~26kD bands Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Hu

Synthetic peptide corresponding to C-DESENSRSPGNKSDN, from human SMN1/SMN2, at the internal region of the protein (NP_075012.1, NP_000335.1).

Recognizes human SMN1/SMN2.

Purified by immunoaffinity chromatography

Supplied as a liquid in Tris saline, 0.02% sodium azide, pH 7.3, 0.5% BSA.

~0.5mg/ml

Product Type: Pab Host: goat Source: human Concentration: ~0.5mg/ml Form: Supplied as a liquid in Tris saline, 0.02% sodium azide, pH 7.3, 0.5% BSA. Purity: Purified by immunoaffinity chromatography. Immunogen: Synthetic peptide corresponding to C-DESENSRSPGNKSDN, from human SMN1/SMN2, at the internal region of the protein (NP_075012.1, NP_000335.1). Specificity: Recognizes human SMN1/SMN2. Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.

E WB

-20°C

1. Setola V, Terao M, Locatelli D, Bassanini S, Garattini E, Battaglia G. Axonal-SMN (a-SMN), a protein isoform of the survival motor neuron gene, is specifically involved in axonogenesis. Proc Natl Acad Sci USA 2007 Feb 6;104(6):1959-64. Epub 2007 Jan 29.

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