SMN1 anticorps (AA 31-100)

N° du produit ABIN1387722

Cet anticorps anti-SMN1 est un anticorps Lapin Polyclonal détectant SMN1 dans WB, ELISA, ICC, IF (cc), IF (p), IHC (p) et IHC (fro). Adapté pour Souris.

Aperçu rapide pour SMN1 anticorps (AA 31-100) (ABIN1387722)

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

Reactivité: Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SMN1 est non-conjugé

Application: Western Blotting (WB), ELISA, Immunocytochemistry (ICC), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Détail du produit anti-SMN1 anticorps

Épitope: AA 31-100

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: WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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: 4 °C,-20 °C

Stockage commentaire: Shipped at 4°C. Store at -20°C for one year. 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

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