BS8018

antibody from Bioworld Technology, Inc

Targeting: SMN2 BCD541, GEMIN1, SMNC, TDRD16B

Western blot (Data presented on provider website)

Immunohistochemistry (Data presented on provider website)

Antibody Data

Product number

BS8018

Provider

Bioworld Technology, Inc

Product name

SMN2 polyclonal antibody

Antibody type

Polyclonal

Antigen

Recombinant full length Human SMN2.

Description

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.

Reactivity

Human, Mouse, Rat

Host

Rabbit

Isotype

IgG

Vial size

100ul

Concentration

1 mg/ml

Storage

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.