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

N° du produit ABIN1391998

Détail du produit anti-SMN1 anticorps (Alexa Fluor 488)

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

Épitope: AA 31-100

Reactivité: Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SMN1 est conjugé à/à la Alexa Fluor 488

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

Réactivité croisée: Souris

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

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human Gemin 1

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

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

Agent conservateur: ProClin

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

Stock: -20 °C

Stockage commentaire: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Date de péremption: 12 months

Détails sur SMN1

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

Autre désignation: Gemin 1/SMA (SMN1 Produits)

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

Sujet:

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