SMN1 Antibody / Survival of Motor Neuron-单抗-深圳欣博盛生物科技有限公司

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SMN1 Antibody / Survival of Motor Neuron

品牌：NSJ Bio

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折扣/货期

货号：V5795-100UG	规格：100 ug	目录价：￥6652.1
货号：V5795-20UG	规格：20 ug	目录价：￥3082.1

产品详情

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产品名称:

SMN1 Antibody / Survival of Motor Neuron

产品描述:

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.

反应种属:

Human

宿主来源:

Mouse

实验应用:

IHC-P

同种型:

Mouse IgG1, kappa

免疫原:

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

克隆性:

Monoclonal (mouse origin)