SMN1 anticorps

N° du produit ABIN7865272

Cet anticorps Souris Monoclonal détecte spécifiquement SMN1 dans IHC. Il présente une réactivité avec des échantillons de Humain.

Aperçu rapide pour SMN1 anticorps (ABIN7865272)

Antigène: SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

Reactivité: Humain

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp SMN1 est non-conjugé

Application: Immunohistochemistry (IHC)

Clone: SMN1-1596

Détail du produit anti-SMN1 anticorps

Fonction: Survival of motor neuron 1, telomeric (SMN1 ) / Gemin 1 Antibody

Specificité: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations of SMN (survival of motor neuron) gene. SMN, also known as Gemin1, SMN1, SMNT and BCD541, exists as four isoforms produced by alternative splicing. SMN is oligomeric and forms a complex with Gemin2 (formerly SIP1), Gemin3 (a DEAD box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. The SMN complex is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (gemini of the coiled bodies). Cytoplasmic SMN interacts with spliceosomal Sm proteins and facilitates their assembly onto U snRNAs, and nuclear SMN mediates recycling of pre-mRNA splicing factors. Nearly identical telomeric and centromeric forms of SMN encode the same protein, however, only mutations in the telomeric form are associated with the disease-state SMA. SMN is expresed in a wide variety of tissues including brain, kidney, liver, spinal cord and moderately in skeletal and cardiac muscle.

Purification: 200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G.

Immunogène: Recombinant full-length human SMN1 protein

Isotype: IgG1, kappa

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Stockage

Concentration: 200 μg/mL

Buffer: Prepared in 10 mM PBS with 0.05 % BSA & 0.05 % azide.

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: 4 °C

Stockage commentaire: Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous. No MSDS required.

Date de péremption: 24 months

Détails sur SMN1

Antigène: SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

Autre désignation: Survival of motor neuron 1, telomeric

Sujet: BTB and CNC homology 1, basic leucine zipper transcription factor 2

Poids moléculaire: 39kDa

ID gène: 6606, 202179

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization