Catalog No.
RHH32804
Species reactivity
Human
Host species
Rabbit
Isotype
IgG
Clonality
Monoclonal
Tested applications
IHC: 1:50-1:100, IP: 1:20, WB: 1:500-1:1000
Target
SMNC, Survival motor neuron protein, SMNT, Component of gems 1, SMN1, SMN, Gemin-1
Concentration
1 mg/ml
Endotoxin level
Please contact with the lab for this information.
Purity
>95% by SDS-PAGE.
Purification
Protein A/G purified from cell culture supernatant.
Accession
Q16637
Applications
IHC, IP, WB
Form
Liquid
Storage buffer
0.01M PBS, pH 7.4, 0.05% BSA, 50% Glycerol, 0.05% Sodium azide.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 4 ℃ for frequent use. Store at -20 ℃ for twelve months from the date of receipt.
Clone ID
R2H29
Epidemiology of Spinal Muscular Atrophy Based on the Results of a Large-Scale Pilot Project on 202,908 Newborns., PMID:38781723
Alberta Spinal Muscular Atrophy Newborn Screening-Results from Year 1 Pilot Project., PMID:37606479
Advances and limitations for the treatment of spinal muscular atrophy., PMID:36329412
Prevalence of Anti-Adeno-Associated Virus Serotype 9 Antibodies in Adult Patients with Spinal Muscular Atrophy., PMID:35943879
Involvement of muscle satellite cell dysfunction in neuromuscular disorders: Expanding the portfolio of satellite cell-opathies., PMID:35302338
[Pharmacological and clinical profile of Onasemnogene Aveparvovec, the first gene therapy for spinal muscular atrophy (SMA)]., PMID:34980814
Activation of Muscle-Specific Kinase (MuSK) Reduces Neuromuscular Defects in the Delta7 Mouse Model of Spinal Muscular Atrophy (SMA)., PMID:34360794
Expert recommendations and clinical considerations in the use of onasemnogene abeparvovec gene therapy for spinal muscular atrophy., PMID:34196026
Specific inhibition of myostatin activation is beneficial in mouse models of SMA therapy., PMID:30481286
Carbon nanofiber-based multiplexed immunosensor for the detection of survival motor neuron 1, cystic fibrosis transmembrane conductance regulator and Duchenne Muscular Dystrophy proteins., PMID:29890394
Electrochemical immunosensors for the detection of survival motor neuron (SMN) protein using different carbon nanomaterials-modified electrodes., PMID:29096367
Cellular bases of the RNA metabolism dysfunction in motor neurons of a murine model of spinal muscular atrophy: Role of Cajal bodies and the nucleolus., PMID:28823932
A novel human-specific splice isoform alters the critical C-terminus of Survival Motor Neuron protein., PMID:27481219
Genome-wide RNA-Seq of Human Motor Neurons Implicates Selective ER Stress Activation in Spinal Muscular Atrophy., PMID:26321202
Systems biology investigation of cAMP modulation to increase SMN levels for the treatment of spinal muscular atrophy., PMID:25514431
KGF and BMP-6 intervene in cellular reprogramming and in mesenchymal-epithelial transition (MET) of 3T3L1 mouse adipose cells., PMID:25492426
Spinal muscular atrophy pathogenic mutations impair the axonogenic properties of axonal-survival of motor neuron., PMID:22324632
Common pathways of autoimmune inflammatory myopathies and genetic neuromuscular disorders., PMID:22083460
Brief report: phenotypic rescue of induced pluripotent stem cell-derived motoneurons of a spinal muscular atrophy patient., PMID:21956898
Double trouble: spinal muscular atrophy type II and seropositive myasthenia gravis in the same patient., PMID:21862330
Proteomic assessment of a cell model of spinal muscular atrophy., PMID:21385431
HuD interacts with survival motor neuron protein and can rescue spinal muscular atrophy-like neuronal defects., PMID:21088113
Abnormal interaction of motor neuropathy-associated mutant HspB8 (Hsp22) forms with the RNA helicase Ddx20 (gemin3)., PMID:20157854
HnRNP C1/C2 may regulate exon 7 splicing in the spinal muscular atrophy gene SMN1., PMID:19628962
Detection of human survival motor neuron (SMN) protein in mice containing the SMN2 transgene: applicability to preclinical therapy development for spinal muscular atrophy., PMID:18771690
Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN-depleted mice., PMID:18521935
[An experimental research on differentiation of mesenchymal stem cells derived from children with spinal muscular atrophy into neuron-like cells]., PMID:17937857
Refined characterization of the expression and stability of the SMN gene products., PMID:17717146
Novel aminoglycosides increase SMN levels in spinal muscular atrophy fibroblasts., PMID:16951947
The benzamide M344, a novel histone deacetylase inhibitor, significantly increases SMN2 RNA/protein levels in spinal muscular atrophy cells., PMID:16724231
A non-sequence-specific requirement for SMN protein activity: the role of aminoglycosides in inducing elevated SMN protein levels., PMID:15790598
Monitoring of recombinant survival motor neuron protein using fiber-optic surface plasmon resonance., PMID:15343403
A survival motor neuron:tetanus toxin fragment C fusion protein for the targeted delivery of SMN protein to neurons., PMID:14644474
Valproic acid increases the SMN2 protein level: a well-known drug as a potential therapy for spinal muscular atrophy., PMID:12915451
Inhibition of apoptosis by Z-VAD-fmk in SMN-depleted S2 cells., PMID:12783893
Survival motor neuron protein in the nucleolus of mammalian neurons., PMID:12126878
A novel association of the SMN protein with two major non-ribosomal nucleolar proteins and its implication in spinal muscular atrophy., PMID:11978761
Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy protein., PMID:11641277
Premature termination mutations in exon 3 of the SMN1 gene are associated with exon skipping and a relatively mild SMA phenotype., PMID:11313744
Expression and subcellular localization of two isoforms of the survival motor neuron protein in different cell types., PMID:11054803
Excitatory amino acid stimulation of the survival of rat cerebellar granule cells in culture is associated with an increase in SMN, the spinal muscular atrophy disease gene product., PMID:10901626
Subcellular localization and axonal transport of the survival motor neuron (SMN) protein in the developing rat spinal cord., PMID:10587577
SMN protein analysis in fibroblast, amniocyte and CVS cultures from spinal muscular atrophy patients and its relevance for diagnosis., PMID:10234506
Subcellular distribution of survival motor neuron (SMN) protein: possible involvement in nucleocytoplasmic and dendritic transport., PMID:9987032
Survival motor neuron (SMN) protein in rat is expressed as different molecular forms and is developmentally regulated., PMID:9758161
Expression of the SMN gene, the spinal muscular atrophy determining gene, in the mammalian central nervous system., PMID:9302277
For research use only. Not for human or drug use.
