Anti-Human SMN1 Monoclonal Antibody

The survival of motor neurons (SMN) genes are the disease genes of spinal muscular atrophy (SMA),a common motor neuron degenerative disease. The level of SMN protein correlates with phenotypic severity of SMA. SMA patients lack a functional SMN1 gene,but they possess an intact SMN2 gene,which though nearly identical to SMN1,is only partially functional,because a large majority of SMN2 transcripts lack exon 7,resulting in production of a truncated,less stable SMN protein. This antibody is specific to human SMN2. It can't recognize mouse and rat SMN.