SMN2 anticorps (N-Term)

N° du produit ABIN2789459

Cet anticorps anti-SMN2 est un anticorps Lapin Polyclonal détectant SMN2 dans WB. Adapté pour Humain, Souris, Chien, Chévre, Cheval, Porc et Lapin.

Aperçu rapide pour SMN2 anticorps (N-Term) (ABIN2789459)

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

Reactivité: Humain, Souris, Chien, Chévre, Cheval, Porc, Lapin

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SMN2 est non-conjugé

Application: Western Blotting (WB)

Détail du produit anti-SMN2 anticorps

Épitope: N-Term

Séquence: KHALKNGDIC ETSGKPKTTP KRKPAKKNKS QKKNTAASLQ QWKVGDKCSA

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

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

Purification: Affinity Purified

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

Information d'application

Indications d'application: Optimal working dilutions should be determined experimentally by the investigator.

Commentaires:

Antigen size: 250 AA

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: Lot specific

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

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.

Conseil sur la manipulation: Avoid repeated freeze-thaw cycles.

Stock: -20 °C

Stockage commentaire: 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.

Détails sur SMN2

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

Autre désignation: SMN2

Sujet: 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

Poids moléculaire: 27 kDa

ID gène: 6607

NCBI Accession: NM_022877, NP_075015

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization