Anti-SMN1/2 Antibody Picoband™ (monoclonal, 2B10) | Boster M03420-1 product information

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 :

Boster

product type :

antibody

product name :

Anti-SMN1/2 Antibody Picoband™ (monoclonal, 2B10)

catalog :

M03420-1

quantity :

100µg/vial

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

2B10

The same clone is also sold as:

Invitrogen: MA5-27878

reactivity :

human, mouse, rat

application :

western blot, immunohistochemistry, immunocytochemistry

product information

SKU :

M03420-1

Product Name :

Anti-SMN1/2 Antibody Picoband™ (monoclonal, 2B10)

Size :

100µg/vial

Clonality :

Monoclonal

Clone Number :

2B10

Host :

Mouse

Reactivity :

Human, Mouse, Rat

Application(s) :

IHC, ICC, WB

Application Details :

Western blot, 0.1-0.5µg/ml. Immunohistochemistry (Paraffin-embedded Section), 0.5-1µg/ml. Immunocytochemistry, 0.5-1µg/ml.

Description :

Boster Bio Anti-SMN1/2 Antibody Picoband™ (monoclonal, 2B10) catalog # M03420-1. Tested in IHC, ICC, WB applications. This antibody reacts with Human, Mouse, Rat.

Concentration :

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

Gene Name :

SMN1

Uniprot ID :

Q16637

Immunogen :

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.

Form :

Lyophilized

Contents :

Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.

Cross-reactivity :

No cross reactivity with other proteins.

Isotype :

IgG1

Storage :

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.

Reconstitution :

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

Gene Full Name :

survival of motor neuron 1, telomeric/survival of motor neuron 2, centromeric

Synonyms :

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

Protein Function :

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. Also required for resolving RNA-DNA hybrids created by RNA polymerase II, that form R-loop in transcription terminal regions, an important step in proper transcription termination. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs).

Subcellular Localization :

Nucleus, gem

Tissue Specificity :

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).

Recommended Detection Systems :

Boster recommends Enhanced Chemiluminescent Kit with anti-Mouse IgG (EK1001) for Western blot, and HRP Conjugated anti-Mouse IgG Super Vision Assay Kit (SV0001-1) for IHC(P) and ICC.

Background :

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.

Research Category :

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