SMN1 anticorps (N-Term)

N° du produit ABIN2778671

Détail du produit anti-SMN1 anticorps

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

Épitope: N-Term

Reactivité: Humain, Souris, Rat, Chien, Porc, Lapin, Cobaye, Cheval

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SMN1 est non-conjugé

Application: Western Blotting (WB)

Séquence: KAVASFKHAL KNGDICETSG KPKTTPKRKP AKKNKSQKKN TAASLQQWKV

Homologie: Dog: 100%, Guinea Pig: 85%, Horse: 93%, Human: 100%, Mouse: 100%, Pig: 93%, Rabbit: 85%, Rat: 92%

Attributs du produit: This is a rabbit polyclonal antibody against SMN1. It was validated on Western Blot using a cell lysate as a positive control.

Purification: Affinity Purified

Immunogène: The immunogen is a synthetic peptide directed towards the N terminal region of human SMN1

Information d'application

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

Commentaires:

Antigen size: 262 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 SMN1

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

Autre désignation: SMN1 (SMN1 Produits)

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

Sujet: 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.
Alias Symbols: BCD541, SMA, SMA1, SMA2, SMA3, SMA4, SMA@, SMN, SMNT, T-BCD541, SMN2
Protein Interaction Partner: COPA, RNF2, BMI1, KDM1A, SMN1, RN7SL1, UBC, HDAC11, MIB1, GEMIN4, DICER1, DDX20, PAN2, RBFOX2, HNRNPUL1, IQCB1, UBL4A, SMN2, NOS2, vpr, GEMIN2, SRP68, SRP54, SRP19, SRP9, RNU2-1, RNU1-1, GEMIN5, MDC1, RBM25, COIL, SRSF5, HSPB1, CUL3, DDAH2, MAST2, SMC5, R
Protein Size: 262

Poids moléculaire: 28 kDa

ID gène: 6606

NCBI Accession: NM_022874, NP_075012

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization