SMN1 anticorps (full length)

N° du produit ABIN7879101

L’anticorps anti-SMN1 Monoclonal Souris est utilisé pour la détection de SMN1 dans des échantillons de Humain. Il a été validé pour IHC (p).

Aperçu rapide pour SMN1 anticorps (full length) (ABIN7879101)

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

Reactivité: Humain

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp SMN1 est non-conjugé

Application: Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Classe de qualité: Carrier-free

Clone: SMN1-1596

Détail du produit anti-SMN1 anticorps

Épitope: full length

Fonction: SMN1 Antibody / Survival of Motor Neuron (azide and preservative free)

Purification: Protein G affinity

Immunogène: A recombinant full-length human SMN1 protein was used as the immunogen for the SMN1 antibody.

Isotype: IgG1, kappa

Information d'application

Indications d'application: Optimal dilution of the SMN1 antibody should be determined by the researcher.

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 mg/mL

Buffer: 1 mg/mL in 1X PBS, BSA free, sodium azide free

Agent conservateur: Azide free

Stock: -20 °C

Stockage commentaire: Aliquot the SMN1 antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Détails sur SMN1

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

Autre désignation: SMN1

Sujet: 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.

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization