SMN Recombinant monoclonal antibody proteintech 86668-2-RR

$449.00
In stock
SKU
86668-2-RR

 

C BCD541, Component of gems 1, Gemin 1, Gemin-1, SMN1

Host / Isotype: Rabbit / IgG Class: Antibody
Reactivity: human, mouse, rat Immunogen: survival of motor neuron 2, centromeric
 Applications: WB, IF/ICC, IP, ELISA Observed Molecular Weight: 38 kDa
Formulation: PBS, Azide, Glycerol GenBank Accession Number: SMN
Conjugate: Unconjugated Gene Symbol: 6607
Tested Applications: Positive WB detected in Gene ID (NCBI): Unconjugated
Application: Western Blot (WB) RRID: Liquid
Dilution: WB : 1:1000-1:4000 Conjugate: Protein A purification
Tested Reactivity: Human, Mouse, Rat Form: Q16637
Host / Isotype: Recombinant Background Information: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of anterior horn cells in the spinal cord and concomitant symmetrical muscle weakness and atrophy (PMID: 16364894 ). SMA is caused by deletion or mutations of the survival motor neuron (SMN1) gene. SMA patients lack a functional SMN1 gene, but they possess an intact SMN2 gene, which though nearly identical to SMN1, is only partially functional (PMID: 17355180). A large majority of SMN2 transcripts lack exon 7, resulting in production of a truncated, less stable SMN protein (PMID: 10369862). The level of SMN protein correlates with phenotypic severity of SMA.

 

 

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