SMN1 Antikörper (AA 31-100) (Alexa Fluor 488)

Produktnummer ABIN1391998

Produktdetails anti-SMN1 Antikörper (Alexa Fluor 488)

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

Bindungsspezifität: AA 31-100

Reaktivität: Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser SMN1 Antikörper ist konjugiert mit Alexa Fluor 488

Applikation: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Kreuzreaktivität: Maus

Homologie: Human,Rat,Dog,Cow,Pig,Rabbit

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human Gemin 1

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu SMN1

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

Andere Bezeichnung: Gemin 1/SMA (SMN1 Produkte)

Synonyme: SMN2 antikoerper, Smn antikoerper, SMN antikoerper, BCD541 antikoerper, GEMIN1 antikoerper, SMA antikoerper, SMA1 antikoerper, SMA2 antikoerper, SMA3 antikoerper, SMA4 antikoerper, SMA@ antikoerper, SMNT antikoerper, T-BCD541 antikoerper, TDRD16A antikoerper, AI849087 antikoerper, Gemin1 antikoerper, SMN1 antikoerper, QtsA-10002 antikoerper, fa12d01 antikoerper, smn antikoerper, wu:fa12d01 antikoerper, survival motor neuron protein antikoerper, survival of motor neuron 1, telomeric antikoerper, survival motor neuron 1 antikoerper, survival of motor neuron 2, centromeric antikoerper, survival motor neuron protein-like antikoerper, survival motor neuron antikoerper, LOC461829 antikoerper, Smn1 antikoerper, SMN1 antikoerper, SMN2 antikoerper, LOC100348318 antikoerper, SMN antikoerper, LOC100713418 antikoerper, LOC100065744 antikoerper, LOC102176643 antikoerper, smn1 antikoerper

Hintergrund:

Synonyms: Component of gems 1, Gemin 1, Gemin-1, Gemin1, SMA 1, SMA 2, SMA 3, SMA 4, SMA, SMA1, SMA2, SMA3, SMA4, SMN 1, SMN, SMN-1, SMN_HUMAN, SMN1, SMN2, SMNT, Survival motor neuron protein, Survival of motor neuron 1 telomeric, survival of motor neuron 1, Survival of motor neuron 1, telomeric, T-BCD541, BCD541, SMN_HUMAN.

Background: 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.

Pathways: Ribonucleoprotein Complex Subunit Organization