04-1466

Anti-SMN Antibody, clone 62E7

clone 62E7, from mouse

• Synonyme(s) : Component of gems 1, gemin 1, spinal muscular atrophy (Werdnig-Hoffmann disease, Kugelberg-Welander disease), survival of motor neuron 1, telomeric
• Code UNSPSC : 12352203
• eCl@ss : 32160702
• Nomenclature NACRES : NA.41

Propriétés

• Source biologique: mouse
• Niveau de qualité: 100
• Forme d'anticorps: purified immunoglobulin
• Type de produit anticorps: primary antibodies
• Clone: 62E7, monoclonal
• Espèces réactives: human
• Réactivité de l'espèce (prédite par homologie): mouse, Xenopus
• Technique(s): immunocytochemistry: suitable; immunoprecipitation (IP): suitable; western blot: suitable
• Isotype: IgG1κ
• Numéro d'accès NCBI: NP_000335.1
• Numéro d'accès UniProt: Q16637
• Conditions d'expédition: wet ice
• Modification post-traductionnelle de la cible: unmodified
• Informations sur le gène: human ... SMA4(11039)

Description

Description générale

The survival of motor neurons (SMN) protein is essential for the biogenesis of small nuclear RNA (snRNA)-ribonucleoproteins (snRNPs), the major components of the pre-mRNA splicing machinery. Though it is ubiquitously expressed, SMN deficiency causes the motor neuron degenerative disease spinal muscular atrophy (SMA). SMN deficiency, similar to that which occurs in severe SMA, has unexpected cell type-specific effects on the repertoire of snRNAs and mRNAs. It alters the stoichiometry of snRNAs and causes widespread pre-mRNA splicing defects in numerous transcripts of diverse genes, preferentially those containing a large number of introns, in SMN-deficient mouse tissues. These findings reveal a key role for the SMN complex in RNA metabolism and in splicing regulation and indicate that SMA is a general splicing disease that is not restricted to motor neurons.

Spécificité

Recognizes SMN.

Immunogène

Epitope: Unknown

His-tagged recombinant protein corresponding to human SMN.

Application

Immunocytochemistry Analysis: 1:500 dilution from a representative lot detected SMN in HeLa cells.

Research Category
Epigenetics & Nuclear Function

Research Sub Category
RNA Metabolism & Binding Proteins

Use Anti-SMN Antibody, clone 62E7 (Mouse Monoclonal Antibody) validated in WB, ICC, IP to detect SMN also known as Spinal muscular atrophy (Werdnig-Hoffmann disease Kugelberg-Welander disease).

Qualité

Evaluated by Western Blot in HeLa cell lysate.

Western Blot Analysis: 0.5 µg/ml of this antibody detected SMN on 10 µg of HeLa cell lysate.

Description de la cible

35 kDa was observed; however, the calculated molecular weight is 31.849 kDa

Forme physique

Format: Purified

Protein G Purified

Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.05% sodium azide.

Stockage et stabilité

Stable for 1 year at 2-8°C from date of receipt.

Remarque sur l'analyse

Control
HeLa cell lysate

Autres remarques

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

Clause de non-responsabilité

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Informations sur la sécurité

• Code de la classe de stockage: 12 - Non Combustible Liquids
• Classe de danger pour l'eau (WGK): WGK 1
• Point d'éclair (°F): Not applicable
• Point d'éclair (°C): Not applicable