SMN2 Antikörper (N-Term)

Produktnummer ABIN2789459

Produktdetails anti-SMN2 Antikörper

Target: SMN2 (Survival of Motor Neuron 2, Centromeric (SMN2))

Bindungsspezifität: N-Term

Reaktivität: Human, Maus, Schwein, Kaninchen, Hund, Ziege, Pferd

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser SMN2 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Sequenz: KHALKNGDIC ETSGKPKTTP KRKPAKKNKS QKKNTAASLQ QWKVGDKCSA

Homologie: Dog: 93%, Goat: 82%, Horse: 92%, Human: 100%, Mouse: 79%, Pig: 86%, Rabbit: 85%

Produktmerkmale: This is a rabbit polyclonal antibody against SMN2. It was validated on Western Blot.

Aufreinigung: Affinity Purified

Immunogen: The immunogen is a synthetic peptide directed towards the N-terminal region of SMN2

Anwendungsinformationen

Applikationshinweise: Optimal working dilutions should be determined experimentally by the investigator.

Kommentare:

Antigen size: 250 AA

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: Lot specific

Buffer: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handhabung: Avoid repeated freeze-thaw cycles.

Lagerung: -20 °C

Informationen zur Lagerung: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.

Details zu SMN2

Target: SMN2 (Survival of Motor Neuron 2, Centromeric (SMN2))

Andere Bezeichnung: SMN2 (SMN2 Produkte)

Synonyme: BCD541 antikoerper, C-BCD541 antikoerper, GEMIN1 antikoerper, SMNC antikoerper, TDRD16B antikoerper, bcd541 antikoerper, c-bcd541 antikoerper, gemin-1 antikoerper, smn2 antikoerper, smnc antikoerper, survival of motor neuron 2, centromeric antikoerper, survival of motor neuron 2, centromeric L homeolog antikoerper, SMN2 antikoerper, smn2.L antikoerper

Hintergrund: 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.
Alias Symbols: BCD541, C-BCD541, FLJ76644, MGC20996, MGC5208, SMN1, SMNC
Protein Interaction Partner: FAM9B, VPS28, BLOC1S6, CHTOP, HNRNPUL1, POLR1C, PPIG, SMN2, BYSL, WWOX, vpr, UBC, SMN1, RBM25, DDX20, COIL, SRSF5, CUL3, POLR2A, GAR1, SNRPG, SNRPF, SNRPB2, TIAL1, DHX9, GEMIN2, SNRPD1, SNRPD3, SNRPD2, SNRPB, FBL, SNRPE, LRIF1, OSTF1, COPS6, LENG8, STXBP3
Protein Size: 250

Molekulargewicht: 27 kDa

Gen-ID: 6607

NCBI Accession: NM_022877, NP_075015

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization