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SMN Polyklonaler Antikörper

SMN Polyklonal Antikörper für WB, IHC, IF/ICC, IP, ELISA

Wirt / Isotyp

Kaninchen / IgG

Getestete Reaktivität

human, Maus, Ratte

Anwendung

WB, IHC, IF/ICC, IP, ELISA

Konjugation

Unkonjugiert

Kat-Nr. : 11708-1-PBS

Synonyme

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



Geprüfte Anwendungen

Produktinformation

11708-1-PBS bindet in WB, IHC, IF/ICC, IP, ELISA SMN und zeigt Reaktivität mit human, Maus, Ratten

Getestete Reaktivität human, Maus, Ratte
Wirt / Isotyp Kaninchen / IgG
Klonalität Polyklonal
Typ Antikörper
Immunogen SMN fusion protein Ag2260
Vollständiger Name survival of motor neuron 2, centromeric
Berechnetes Molekulargewicht 282 aa, 30 kDa
Beobachtetes Molekulargewicht 38 kDa
GenBank-ZugangsnummerBC000908
Gene symbol SMN
Gene ID (NCBI) 6607
Konjugation Unkonjugiert
Form Liquid
Reinigungsmethode Antigen-Affinitätsreinigung
Lagerungspuffer PBS only
LagerungsbedingungenStore at -80°C. 20ul Größen enthalten 0,1% BSA.

Hintergrundinformationen

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. This antibody, 11708-1-AP, raised against the recombinant full-length human SMN2 protein, recognizes all isoforms of SMN protein.