antibody

Anti-SMN1/2 Antibody Picoband™

PB9398

100μg/vial

polyclonal

domestic rabbit

nonconjugated

human, mouse, rat

western blot, immunocytochemistry, flow cytometry, immunohistochemistry - paraffin section

Anti-SMN1/2 Antibody Picoband™

100μg/vial

Polyclonal

Rabbit

Human, Mouse, Rat

Hamster

Flow Cytometry, IF, IHC-P, ICC, WB

Western blot, 0.1-0.5µg/ml, Human, Mouse, Rat . Immunohistochemistry (Paraffin-embedded Section), 0.5-1µg/ml, Human, Mouse, Rat, By Heat. Immunocytochemistry/Immunofluorescence, 2µg/ml, Human. Flow Cytometry, 1-3μg/1x10^6 cells, Human.

WB: The detection limit for SMN1/2 is approximately 0.1ng/lane under reducing conditions. Tested Species: In-house tested species with positive results. By Heat: Boiling the paraffin sections in 10mM citrate buffer, pH6.0, for 20mins is required for the staining of formalin/paraffin sections. Other applications have not been tested. Optimal dilutions should be determined by end users.

Boster Bio Anti-SMN1/2 Antibody Picoband™ catalog # PB9398. Tested in Flow Cytometry, IF, IHC-P, ICC, WB applications. This antibody reacts with Human, Mouse, Rat.

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

SMN1

Q16637

A synthetic peptide corresponding to a sequence at the N-terminus of human SMN1/2 (22-52aa RRGTGQSDDSDIWDDTALIKAYDKAVASFKH), identical to the related mouse and rat sequences.

Lyophilized

Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.

Immunogen affinity purified.

No cross reactivity with other proteins.

Store at -20˚C for one year from date of receipt. After reconstitution, at 4˚C for one month. It can also be aliquotted and stored frozen at -20˚C for six months. Avoid repeated freeze-thaw cycles.

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Survival motor neuron protein

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

Survival motor neuron protein

31849 MW

The SMN complex plays a catalyst role in the assembly of small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome. Thereby, plays an important role in the splicing of cellular pre-mRNAs. Most spliceosomal snRNPs contain a common set of Sm proteins SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE, SNRPF and SNRPG that assemble in a heptameric protein ring on the Sm site of the small nuclear RNA to form the core snRNP. In the cytosol, the Sm proteins SNRPD1, SNRPD2, SNRPE, SNRPF and SNRPG are trapped in an inactive 6S pICln-Sm complex by the chaperone CLNS1A that controls the assembly of the core snRNP. Dissociation by the SMN complex of CLNS1A from the trapped Sm proteins and their transfer to an SMN-Sm complex triggers the assembly of core snRNPs and their transport to the nucleus. Ensures the correct splicing of U12 intron-containing genes that may be important for normal motor and proprioceptive neurons development. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs).

Cytoplasm. Nucleus, gem. Nucleus, Cajal body. Cytoplasmic granule. Cytoplasm, myofibril, sarcomere, Z line. Colocalizes with Actn at the Z-line of skeletal muscle (By similarity). Under stress conditions colocalizes with RPP20/POP7 in punctuated cytoplasmic granules. Colocalized and redistributed with ZPR1 from the cytoplasm to nuclear gems (Gemini of coiled bodies) and Cajal bodies.

Expressed in a wide variety of tissues. Expressed at high levels in brain, kidney and liver, moderate levels in skeletal and cardiac muscle, and low levels in fibroblasts and lymphocytes. Also seen at high levels in spinal cord. Present in osteoclasts and mononuclear cells (at protein level).

Boster recommends Enhanced Chemiluminescent Kit with anti-Rabbit IgG (EK1002) for Western blot, and HRP Conjugated anti-Rabbit IgG Super Vision Assay Kit (SV0002-1) for IHC(P) and ICC.

Belongs to the SMN family.

This gene is part of a 500 kb 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. However, mutations in this gene, the telomeric copy, are associated with spinal muscular atrophy; mutations in the centromeric copy do not lead to disease. The centromeric copy 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. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The 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. Multiple transcript variants encoding distinct isoforms have been described.

DNA / RNA, Epigenetics and Nuclear Signaling, Neural Signal Transduction, Neurology Process, Neuroscience, RNA Processing, Splicing