SMN1 Antikörper (full length)

Produktnummer ABIN7879102

Der Maus Monoklonal anti-SMN1 Antikörper (Klon SMN1-1596) (ABIN7879102) detektiert spezifisch SMN1 in IHC (p). Dieser Antikörper reagiert spezifisch mit Proben aus Human.

Kurzübersicht für SMN1 Antikörper (full length) (ABIN7879102)

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

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser SMN1 Antikörper ist unkonjugiert

Applikation: Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Klon: SMN1-1596

Produktdetails anti-SMN1 Antikörper

Bindungsspezifität: full length

Verwendungszweck: SMN1 Antibody / Survival of Motor Neuron

Aufreinigung: Protein G affinity

Immunogen: A recombinant full-length human SMN1 protein was used as the immunogen for the SMN1 antibody.

Isotyp: IgG1, kappa

Anwendungsinformationen

Applikationshinweise: Optimal dilution of the SMN1 antibody should be determined by the researcher.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 0.2 mg/mL

Buffer: 0.2 mg/mL in 1X PBS with 0.1 mg/mL BSA (US sourced), 0.05 % sodium azide

Konservierungsmittel: Sodium azide

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

Lagerung: -20 °C

Informationen zur Lagerung: Aliquot the SMN1 antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Details zu SMN1

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

Andere Bezeichnung: SMN1

Hintergrund: 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.

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization