SMN-Exon7 Monoklonaler Antikörper

SMN-Exon7 Monoklonal Antikörper für WB, IHC, IF/ICC, Indirect ELISA

Wirt / Isotyp

Maus / IgG1

Getestete Reaktivität

human, Maus, Ratte

Anwendung

WB, IHC, IF/ICC, Indirect ELISA

Konjugation

Unkonjugiert

CloneNo.

3A8G11

Kat-Nr. : 60255-1-PBS

Synonyme

SMN, 3A8G11, Component of gems 1, Gemin 1, Gemin-1



Geprüfte Anwendungen

Produktinformation

60255-1-PBS bindet in WB, IHC, IF/ICC, Indirect ELISA SMN-Exon7 und zeigt Reaktivität mit human, Maus, Ratten

Getestete Reaktivität human, Maus, Ratte
Wirt / Isotyp Maus / IgG1
Klonalität Monoklonal
Typ Antikörper
Immunogen SMN-Exon7 fusion protein Ag16615
Vollständiger Name survival of motor neuron 1, telomeric
Berechnetes Molekulargewicht 294 aa, 32 kDa
Beobachtetes Molekulargewicht 40 kDa
GenBank-ZugangsnummerBC062723
Gene symbol SMN
Gene ID (NCBI) 6606
Konjugation Unkonjugiert
Form Liquid
Reinigungsmethode Protein-G-Reinigung
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, 60255-1-Ig, raised against the C-terminal region (275-294aa) encoded by the exon 7.