SMN1 Antikörper

Produktnummer ABIN7865272

Dieser Maus Monoklonal Antikörper detektiert spezifisch SMN1 in IHC. Es zeigt Reaktivität gegenüber Proben von Human.

Kurzübersicht für SMN1 Antikörper (ABIN7865272)

Target: SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser SMN1 Antikörper ist unkonjugiert

Applikation: Immunohistochemistry (IHC)

Klon: SMN1-1596

Produktdetails anti-SMN1 Antikörper

Verwendungszweck: Survival of motor neuron 1, telomeric (SMN1 ) / Gemin 1 Antibody

Spezifität: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations of SMN (survival of motor neuron) gene. SMN, also known as Gemin1, SMN1, SMNT and BCD541, exists as four isoforms produced by alternative splicing. SMN is oligomeric and forms a complex with Gemin2 (formerly SIP1), Gemin3 (a DEAD box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. The SMN complex is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (gemini of the coiled bodies). Cytoplasmic SMN interacts with spliceosomal Sm proteins and facilitates their assembly onto U snRNAs, and nuclear SMN mediates recycling of pre-mRNA splicing factors. Nearly identical telomeric and centromeric forms of SMN encode the same protein, however, only mutations in the telomeric form are associated with the disease-state SMA. SMN is expresed in a wide variety of tissues including brain, kidney, liver, spinal cord and moderately in skeletal and cardiac muscle.

Aufreinigung: 200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G.

Immunogen: Recombinant full-length human SMN1 protein

Isotyp: IgG1, kappa

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Konzentration: 200 μg/mL

Buffer: Prepared in 10 mM PBS with 0.05 % BSA & 0.05 % azide.

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: 4 °C

Informationen zur Lagerung: Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous. No MSDS required.

Haltbarkeit: 24 months

Details zu SMN1

Target: SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

Andere Bezeichnung: Survival of motor neuron 1, telomeric

Hintergrund: BTB and CNC homology 1, basic leucine zipper transcription factor 2

Molekulargewicht: 39kDa

Gen-ID: 6606, 202179

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization