Anti-SMN1 Antibody (R2H29)

Catalog No.

RHH32804

Species reactivity

Human

Host species

Rabbit

Isotype

IgG

Clonality

Monoclonal

Applications

IHC, IP, WB

Recommended Dilution

Application	Dilution
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

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

Note

For research use only.

Expression of the SMN gene, the spinal muscular atrophy determining gene, in the mammalian central nervous system., PMID:9302277

Survival motor neuron (SMN) protein in rat is expressed as different molecular forms and is developmentally regulated., PMID:9758161

Subcellular distribution of survival motor neuron (SMN) protein: possible involvement in nucleocytoplasmic and dendritic transport., PMID:9987032

SMN protein analysis in fibroblast, amniocyte and CVS cultures from spinal muscular atrophy patients and its relevance for diagnosis., PMID:10234506

Subcellular localization and axonal transport of the survival motor neuron (SMN) protein in the developing rat spinal cord., PMID:10587577

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

Expression and subcellular localization of two isoforms of the survival motor neuron protein in different cell types., PMID:11054803

Premature termination mutations in exon 3 of the SMN1 gene are associated with exon skipping and a relatively mild SMA phenotype., PMID:11313744

Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy protein., PMID:11641277

A novel association of the SMN protein with two major non-ribosomal nucleolar proteins and its implication in spinal muscular atrophy., PMID:11978761

Survival motor neuron protein in the nucleolus of mammalian neurons., PMID:12126878

Inhibition of apoptosis by Z-VAD-fmk in SMN-depleted S2 cells., PMID:12783893

Valproic acid increases the SMN2 protein level: a well-known drug as a potential therapy for spinal muscular atrophy., PMID:12915451

A survival motor neuron:tetanus toxin fragment C fusion protein for the targeted delivery of SMN protein to neurons., PMID:14644474

Monitoring of recombinant survival motor neuron protein using fiber-optic surface plasmon resonance., PMID:15343403

A non-sequence-specific requirement for SMN protein activity: the role of aminoglycosides in inducing elevated SMN protein levels., PMID:15790598

The benzamide M344, a novel histone deacetylase inhibitor, significantly increases SMN2 RNA/protein levels in spinal muscular atrophy cells., PMID:16724231

Novel aminoglycosides increase SMN levels in spinal muscular atrophy fibroblasts., PMID:16951947

Refined characterization of the expression and stability of the SMN gene products., PMID:17717146

[An experimental research on differentiation of mesenchymal stem cells derived from children with spinal muscular atrophy into neuron-like cells]., PMID:17937857

Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN-depleted mice., PMID:18521935

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

HnRNP C1/C2 may regulate exon 7 splicing in the spinal muscular atrophy gene SMN1., PMID:19628962

Abnormal interaction of motor neuropathy-associated mutant HspB8 (Hsp22) forms with the RNA helicase Ddx20 (gemin3)., PMID:20157854

HuD interacts with survival motor neuron protein and can rescue spinal muscular atrophy-like neuronal defects., PMID:21088113

Proteomic assessment of a cell model of spinal muscular atrophy., PMID:21385431

Double trouble: spinal muscular atrophy type II and seropositive myasthenia gravis in the same patient., PMID:21862330

Brief report: phenotypic rescue of induced pluripotent stem cell-derived motoneurons of a spinal muscular atrophy patient., PMID:21956898

Common pathways of autoimmune inflammatory myopathies and genetic neuromuscular disorders., PMID:22083460

Spinal muscular atrophy pathogenic mutations impair the axonogenic properties of axonal-survival of motor neuron., PMID:22324632

KGF and BMP-6 intervene in cellular reprogramming and in mesenchymal-epithelial transition (MET) of 3T3L1 mouse adipose cells., PMID:25492426

Systems biology investigation of cAMP modulation to increase SMN levels for the treatment of spinal muscular atrophy., PMID:25514431

Genome-wide RNA-Seq of Human Motor Neurons Implicates Selective ER Stress Activation in Spinal Muscular Atrophy., PMID:26321202

A novel human-specific splice isoform alters the critical C-terminus of Survival Motor Neuron protein., PMID:27481219

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

Electrochemical immunosensors for the detection of survival motor neuron (SMN) protein using different carbon nanomaterials-modified electrodes., PMID:29096367

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

Specific inhibition of myostatin activation is beneficial in mouse models of SMA therapy., PMID:30481286

Expert recommendations and clinical considerations in the use of onasemnogene abeparvovec gene therapy for spinal muscular atrophy., PMID:34196026

Activation of Muscle-Specific Kinase (MuSK) Reduces Neuromuscular Defects in the Delta7 Mouse Model of Spinal Muscular Atrophy (SMA)., PMID:34360794

[Pharmacological and clinical profile of Onasemnogene Aveparvovec, the first gene therapy for spinal muscular atrophy (SMA)]., PMID:34980814

Involvement of muscle satellite cell dysfunction in neuromuscular disorders: Expanding the portfolio of satellite cell-opathies., PMID:35302338

Prevalence of Anti-Adeno-Associated Virus Serotype 9 Antibodies in Adult Patients with Spinal Muscular Atrophy., PMID:35943879

Advances and limitations for the treatment of spinal muscular atrophy., PMID:36329412

Alberta Spinal Muscular Atrophy Newborn Screening-Results from Year 1 Pilot Project., PMID:37606479

Epidemiology of Spinal Muscular Atrophy Based on the Results of a Large-Scale Pilot Project on 202,908 Newborns., PMID:38781723

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