SMN1 anticorps

N° du produit ABIN6568829

Cet anticorps Lapin Polyclonal détecte spécifiquement SMN1 dans WB et IHC. Il présente une réactivité envers Humain, Souris et Rat.

Aperçu rapide pour SMN1 anticorps (ABIN6568829)

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

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SMN1 est non-conjugé

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

Détail du produit anti-SMN1 anticorps

Purification: Affinity purification

Immunogène: Recombinant protein of human SMN2

Isotype: IgG

Information d'application

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

Restrictions: For Research Use only

Stockage

Concentration: 1 mg/mL

Buffer: 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:

Synonyms: BCD541,Component of gems 1,Gemin 1,Gemin-1,OTTHUMP00000125198,OTTHUMP00000223567,OTTHUMP00000223568,OTTHUMP00000224066,OTTHUMP00000226924,SMA 1,SMA 2,SMA 3,SMA 4,SMA,SMA@,SMA1,SMA2,SMA3,SMA4,SMN,SMN,SMN1,SMN2,SMNT,Survival motor neuron protein,Survival of motor neuron 1,telomeric,T-BCD541

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. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. This gene 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 full length 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. Four transcript variants encoding distinct isoforms have been described.

Poids moléculaire:

Observed_MW: 35kDa

Calculated_MW: 27kDa/28kDa/30kDa/31kDa

ID gène: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization