Anti-SMN2 Antibody (N179/8)

Catalog No.

RHH32803

Host species

Mouse

Isotype

IgG2a, kappa

Clonality

Monoclonal

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% as determined by SDS-PAGE.

Purification

Protein A/G purified from cell culture supernatant.

Applications

IHC, WB

Form

Liquid

Storage buffer

0.01M PBS, pH 7.4.

Stability and Storage

Use a manual defrost freezer and avoid repeated freeze-thaw cycles. Store at 4°C short term (1-2 weeks). Store at -20°C 12 months. Store at -80°C long term.

Clone ID

N179/8

Advances in Disease-Modifying Therapeutics for Chronic Neuromuscular Disorders., PMID:39708835

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

Repeated AAV9 Titer Determination in a Presymptomatic SMA Patient with Three SMN2 Gene Copies - A Case Report., PMID:38306058

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

Antibody-oligonucleotide conjugate achieves CNS delivery in animal models for spinal muscular atrophy., PMID:36346674

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

Newborn Screening for Spinal Muscular Atrophy in New York State: Clinical Outcomes From the First 3 Years., PMID:35835557

Adeno-associated virus serotype 9 antibody titers in patients with SMA pre-screened for treatment with onasemnogene abeparvovec -routine care evidence., PMID:35474244

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

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

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

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

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

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

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

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