SMN1 anticorps (AA 80-150)

N° du produit ABIN7270652

L’anticorps Lapin Polyclonal anti-SMN1 a été validé pour WB et IHC. Il convient pour détecter SMN1 dans des échantillons de Humain.

Aperçu rapide pour SMN1 anticorps (AA 80-150) (ABIN7270652)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SMN1 est non-conjugé

Application: Western Blotting (WB), Immunohistochemistry (IHC)

Détail du produit anti-SMN1 anticorps

Épitope: AA 80-150

Fonction: SMN1 Rabbit pAb

Séquence: SQKKNTAASL QQWKVGDKCS AIWSEDGCIY PATIASIDFK RETCVVVYTG YGNREEQNLS DLLSPICEVA N

Réactivité croisée: Humain, Souris, Rat

Attributs du produit: Polyclonal Antibodies

Purification: Affinity purification

Immunogène: Recombinant fusion protein containing a sequence corresponding to amino acids 80-150 of human SMN1 (NP_000335.1).

Isotype: IgG

Information d'application

Indications d'application: WB,1:500 - 1:2000,IHC,1:50 - 1:200

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: PBS with 0.02 % sodium azide,50 % glycerol, pH 7.3.

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: -20 °C

Stockage commentaire: Store at -20°C. Avoid freeze / thaw cycles.

Détails sur SMN1

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

Autre désignation: SMN1

Sujet: This gene is part of a 500 kb inverted duplication on chromosome 5q13. 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. 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.,SMN1,BCD541,GEMIN1,SMA,SMA1,SMA2,SMA3,SMA4,SMN1

Poids moléculaire: 27kDa/28kDa/30kDa/31kDa

ID gène: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization