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anti-Mouse (Murine) SMN1 Antikörper:
anti-Human SMN1 Antikörper:
anti-Rat (Rattus) SMN1 Antikörper:
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Mouse (Murine) Monoclonal SMN1 Primary Antibody für ICC, FACS - ABIN108567
Liu, Dreyfuss: A novel nuclear structure containing the survival of motor neurons protein. in The EMBO journal 1996
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Mouse (Murine) Monoclonal SMN1 Primary Antibody für ICC, FACS - ABIN108566
Grondard, Biondi, Armand, Lécolle, Della Gaspera, Pariset, Li, Gallien, Vidal, Chanoine, Charbonnier: Regular exercise prolongs survival in a type 2 spinal muscular atrophy model mouse. in The Journal of neuroscience : the official journal of the Society for Neuroscience 2005
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Human Monoclonal SMN1 Primary Antibody für IF, IHC - ABIN967053
Hendrickson, Donohoe, Akmaev, Sugarman, Labrousse, Boguslavskiy, Flynn, Rohlfs, Walker, Allitto, Sears, Scholl: Differences in SMN1 allele frequencies among ethnic groups within North America. in Journal of medical genetics 2009
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Human Monoclonal SMN1 Primary Antibody für FACS, IHC - ABIN967052
Martins de Araújo, Bonnal, Hastings, Krainer, Valcárcel: Differential 3' splice site recognition of SMN1 and SMN2 transcripts by U2AF and U2 snRNP. in RNA (New York, N.Y.) 2009
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Human Polyclonal SMN1 Primary Antibody für IHC, ELISA - ABIN1585252
Smith, Kuliszewski, Liao, Rudenko, Stewart, Leong-Poi: Sustained improvement in perfusion and flow reserve after temporally separated delivery of vascular endothelial growth factor and angiopoietin-1 plasmid deoxyribonucleic acid. in Journal of the American College of Cardiology 2012
Human Monoclonal SMN1 Primary Antibody für IHC, ELISA - ABIN1585251
Zhu, Guo, Yao, Yan, Xue, Hao, Zhou, Zhu, Qin, Lu: Synergy between Kaposi's sarcoma-associated herpesvirus (KSHV) vIL-6 and HIV-1 Nef protein in promotion of angiogenesis and oncogenesis: role of the AKT signaling pathway. in Oncogene 2014
Data show that the coding sequence of survival of motor neuron 2 (SMN2) differs from that of survival motor neuron 1 (SMN1) by a single nucleotide (c.840C>T) at codon 280 in exon 7.
SMN (zeige SNRPN Antikörper) protein functions in cytoplasmic Sm-core assembly and in the recruitment of the snRNA cap hypermethylase
miR (zeige MLXIP Antikörper)-431 expression was highly increased, and a number of its putative mRNA targets were significantly downregulated in motor neurons after SMN (zeige STMN1 Antikörper) loss. Further, we found that miR (zeige MLXIP Antikörper)-431 regulates motor neuron neurite length by targeting several molecules previously identified to play a role in motor neuron axon outgrowth, including chondrolectin (zeige CHODL Antikörper)
To determine the dependence of oligodendrocyte (OL)on the Smn (zeige STMN1 Antikörper) protein(SMN1), we utilized the Smn (zeige STMN1 Antikörper)-/-;SMN2 (severe) mouse model. Our data suggest that despite the multi-functionality and ubiquitous expression of the Smn (zeige STMN1 Antikörper) protein, it does not play a key role in myelination of the CNS, at least in the context of spinal muscular atrophy pathogenesis.
our studies show that this G-motif represents a novel and essential determinant for axonal localization of the Anxa2 (zeige ANXA2 Antikörper) mRNA mediated by the SMN (zeige STMN1 Antikörper) complex.
A long non-coding RNA (lncRNA) that arises from the antisense strand of SMN (zeige STMN1 Antikörper), SMN (zeige STMN1 Antikörper)-AS1 (zeige ARSB Antikörper), is enriched in neurons and transcriptionally represses SMN (zeige STMN1 Antikörper) expression by recruiting the epigenetic Polycomb (zeige CBX2 Antikörper) repressive complex-2.
SMN1 expression restoration is curative in a spinal muscular atrophy model mice.
Survival motor neuron 1, and survival motor neuron 2, depletion results in increased alternative splicing events.
these results demonstrate that SMN (zeige STMN1 Antikörper) deficiency impacts spleen development and suggests a potential role for immunological development in Spinal muscular atrophy.
Itch monoubiquitinates SMN (zeige STMN1 Antikörper) and monoubiquitination of SMN (zeige STMN1 Antikörper) plays an important role in regulating its cellular localization.
muscle does not appear to require high levels of SMN (zeige STMN1 Antikörper) above what is produced by two copies of SMN2
Findings demonstrate that high expression of SMN (zeige STMN1 Antikörper) in the motor neuron is both necessary and sufficient for proper function of the motor unit. In addition, SMN (zeige STMN1 Antikörper) high expression in neurons and glia has a major impact on survival.
Mutation in SMN1 is associated with Spinal Muscular Atrophy.
Research has shown that SMN (zeige STMN1 Antikörper), both on the mRNA and protein level, is highly affected by cellular stress. In this review we will summarize the research that highlights the roles of SMN (zeige STMN1 Antikörper) in the disease process and the response of SMN (zeige STMN1 Antikörper) to various environmental stresses.
Ongoing research may yield other treatments, especially for children who have not responded to Spinraza. A gene therapy delivered by adeno (zeige ADORA2A Antikörper)-associated virus type 9 (AAV9) is designed to replace or correct SMN1 . Cure SMA is supporting research in this area as well as studies of small molecules that correct SMN2 splicing or spur it to produce more protein.
In lesional SSc (zeige CYP11A1 Antikörper) dermal fibroblasts, GKT-137831 reduced alpha-SMA and CCN2 (zeige CTGF Antikörper) protein overexpression and collagen gel contraction
Carrier risks for individuals having two copies of SMN1 in SMA families with 2-copy alleles were deduced. A meta-analysis including large sample sizes from the Chinese population was performed in order to generate a solid data basis for this calculation.
From the computational analysis, it is also possible that SMN's Lys45 and Asp36 act as two electrostatic clips at the SMN (zeige STMN1 Antikörper)-Gemin2 (zeige GEMIN2 Antikörper) complex structure interface
Loss of SMN1 is associated with Spinal muscular atrophy.
A rare variant in exon 7 of SMN1 in three patients affected with type I or type II SMA. Most of the SMN1 transcripts exhibited complete loss of exon 7 in vivo. The variant disrupts Tra2beta1 binding.
These results establish that SMN overexpression in motor neurons slows disease onset and outcome by ameliorating pathological signs in this model of mutant TDP-43-mediated amyotrophic lateral sclerosis (ALS).
first cloning and identification of the swine SMN1 gene and show that there is significant sequence homology between swine and human SMN (zeige SNRPN Antikörper) throughout the coding region
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. 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 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 encoding distinct isoforms have been described.
survival of motor neuron 2, centromeric
, survival motor neuron protein
, ATP-dependent helicase IGHMBP2
, DNA-binding protein SMUBP-2
, antifreeze enhancer-binding protein
, cardiac transcription factor 1
, immunoglobulin S mu binding protein 2
, immunoglobulin mu-binding protein 2
, neuromuscular degeneration
, p110 subunit
, survival of motor neuron protein
, component of gems 1
, survival motor neuron 1 protein
, tudor domain containing 16A
, survival motor neuron 1