Anti-SMN2 [SMN-KH]

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References (2)

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Applications	IHC WB
Antigen/Gene or Protein Targets	SMN2
Reactivity	Human
Relevance	Survival motor neuron (SMN) protein is expressed from two linked paralogous genes: Survival of motor neuron 1, telomeric (SMN1) and Survival of motor neuron 2, centromeric (SMN2). SMNs are primarily localized in the cytoplasm and nuclear gems of all cells, where they are understood to mediate the assembly of spliceosomal small nuclear ribonucleoprotein particles (snRNPs). The progressive loss of functional SMNs in the anterior horn of the spinal cord is a critical cause of Spinal Muscular Atrophy (SMA), an autosomal recessive neuromuscular disease. There are three known types of childhood-onset SMA, in addition to a fourth type that is characterized by adult-onset SMA.
Host	Mouse
Immunogen	MBP-tagged recombinant protein corresponding to human SMN2
Subclass	IgG1 kappa
Research Area	Epigenetics & Nuclear Signalling, Neurobiology

There are 2 reference entries for this reagent.

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Hua et al. 2010. Genes Dev. 24(15):1634-44. PMID: 20624852.

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Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model.

Europe PMC ID: 20624852

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Hua et al. 2010. Genes Dev. 24(15):1634-44. PMID: 20624852.

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Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model.

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	Cold Spring Harbor Laboratory
	14 reagents listed on Ximbio