SMN1 Antikörper (Survival of Motor Neuron 1, Telomeric)

Details for Product anti-SMN1 Antibody No. ABIN6567523
Target
Reaktivität
Human, Maus, Ratte
70
28
27
5
5
3
3
2
2
1
1
1
Wirt
Kaninchen
56
13
1
Klonalität
Polyklonal
Konjugat
Dieser SMN1 Antikörper ist unkonjugiert
6
4
4
3
2
2
2
2
2
2
2
2
1
1
1
1
1
Applikation
Immunohistochemistry (IHC), Western Blotting (WB)
50
30
13
10
9
7
5
4
4
1
1
1
Optionen
Immunogen Recombinant protein of human SMN2
Isotyp IgG
Reinigung Affinity purification
Target
Andere Bezeichnung SMN1 (SMN1 Antibody Abstract)
Hintergrund

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.

Molekulargewicht

Observed_MW: 35kDa

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

Gen-ID 6606
UniProt Q16637
Pathways Ribonucleoprotein Complex Subunit Organization
Applikations-hinweise WB 1:500 - 1:2000 IHC 1:50 - 1:200
Beschränkungen Nur für Forschungszwecke einsetzbar
Konzentration 1 mg/mL
Buffer Buffer: PBS with 0.02 % sodium azide, 50 % glycerol,  pH 7.3.
Konservierungs-mittel Sodium azide
Vorsichtsmaßnahmen This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Lagerung -20 °C
Informationen zur Lagerung Store at -20°C. Avoid freeze / thaw cycles.
Bilder
Image no. 1 for anti-Survival of Motor Neuron 1, Telomeric (SMN1) antibody (ABIN6567523) Immunohistochemistry of paraffin-embedded human prostate using SMN2 antibody.
Image no. 2 for anti-Survival of Motor Neuron 1, Telomeric (SMN1) antibody (ABIN6567523) Immunohistochemistry of paraffin-embedded rat heart using SMN2 antibody.
Image no. 3 for anti-Survival of Motor Neuron 1, Telomeric (SMN1) antibody (ABIN6567523) Western blot analysis of extracts of various cell lines, using SMN2 antibody.