SMN1 Antikörper

Produktnummer ABIN2462049

Produktdetails anti-SMN1 Antikörper

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

Reaktivität: Human, Hund

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser SMN1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA

Aufreinigung: Antibody is purified by peptide affinity chromatography method.

Immunogen: Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human SMN1.

Anwendungsinformationen

Applikationshinweise: SMN1 antibody can be used for detection of SMN1 by ELISA at 1:62500. SMN1 antibody can be used for detection of SMN1 by western blot at 0.5 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Add 50 ?L of distilled water. Final antibody concentration is 1 mg/mL.

Konzentration: 1 mg/mL

Buffer: Antibody is lyophilized in PBS buffer with 2 % sucrose.

Handhabung: As with any antibody avoid repeat freeze-thaw cycles.

Lagerung: 4 °C/-20 °C

Informationen zur Lagerung: For short periods of storage (days) store at 4 °C. For longer periods of storage, store SMN1 antibody at -20 °C.

Details zu SMN1

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

Andere Bezeichnung: SMN1 (SMN1 Produkte)

Synonyme: SMN2 antikoerper, Smn antikoerper, SMN antikoerper, BCD541 antikoerper, GEMIN1 antikoerper, SMA antikoerper, SMA1 antikoerper, SMA2 antikoerper, SMA3 antikoerper, SMA4 antikoerper, SMA@ antikoerper, SMNT antikoerper, T-BCD541 antikoerper, TDRD16A antikoerper, AI849087 antikoerper, Gemin1 antikoerper, SMN1 antikoerper, QtsA-10002 antikoerper, fa12d01 antikoerper, smn antikoerper, wu:fa12d01 antikoerper, survival motor neuron protein antikoerper, survival of motor neuron 1, telomeric antikoerper, survival motor neuron 1 antikoerper, survival of motor neuron 2, centromeric antikoerper, survival motor neuron protein-like antikoerper, survival motor neuron antikoerper, LOC461829 antikoerper, Smn1 antikoerper, SMN1 antikoerper, SMN2 antikoerper, LOC100348318 antikoerper, SMN antikoerper, LOC100713418 antikoerper, LOC100065744 antikoerper, LOC102176643 antikoerper, smn1 antikoerper

Hintergrund: SMN1 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.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. 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. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. 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. Two transcript variants are produced by this gene.

Molekulargewicht: 28 kDa

Gen-ID: 6606

NCBI Accession: NP_075012

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization