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SMN1 抗体 (full length)

The 小鼠 单克隆 anti-SMN1 antibody is suitable to detect SMN1 in samples from 人. It has been validated for IHC (p).
产品编号 ABIN7879101
发货至: 中国
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Quick Overview for SMN1 抗体 (full length) (ABIN7879101)

抗原

See all SMN1 抗体
SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

适用

  • 75
  • 44
  • 26
  • 6
  • 6
  • 3
  • 3
  • 2
  • 2
  • 1
  • 1
  • 1

宿主

  • 75
  • 15
小鼠

克隆类型

  • 55
  • 35
单克隆

标记

  • 44
  • 6
  • 4
  • 3
  • 3
  • 3
  • 3
  • 3
  • 3
  • 3
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
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This SMN1 antibody is un-conjugated

应用范围

  • 67
  • 27
  • 26
  • 20
  • 13
  • 13
  • 13
  • 9
  • 8
  • 5
  • 3
  • 1
  • 1
Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

质量等级

Carrier-free

克隆位点

SMN1-1596
  • 抗原表位

    • 15
    • 9
    • 5
    • 5
    • 4
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    full length

    原理

    SMN1 Antibody / Survival of Motor Neuron (azide and preservative free)

    纯化方法

    Protein G affinity

    免疫原

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

    亚型

    IgG1, kappa
  • 应用备注

    Optimal dilution of the SMN1 antibody should be determined by the researcher.

    限制

    仅限研究用
  • 状态

    Liquid

    浓度

    1 mg/mL

    缓冲液

    1 mg/mL in 1X PBS, BSA free, sodium azide free

    储存液

    Azide free

    储存条件

    -20 °C

    储存方法

    Aliquot the SMN1 antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
  • 抗原

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

    别名

    SMN1

    背景

    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.

    UniProt

    Q16637

    途径

    Ribonucleoprotein Complex Subunit Organization
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