Survival of motor neuron 1; telomeric (SMN1 ) / Gemin 1 Antibody
Product Sizes
20 ug
£364.00
6606-MSM1-P0-20UG
About this Product
- SKU:
- 6606-MSM1-P0
- Additional Names:
- BCD541 antibody; Component of gems 1 antibody; Gemin 1 antibody; Gemin-1 antibody; OTTHUMP00000125198 antibody; OTTHUMP00000223567 antibody; OTTHUMP00000223568 antibody; OTTHUMP00000224066 antibody; OTTHUMP00000226924 antibody; SMA 1 antibody; SMA 2 antibody; SMA 3 antibody; SMA 4 antibody; SMA antibody SMA@ antibody SMA1 antibody SMA2 antibody SMA3 antibody SMA4 antibody SMN antibody SMN_HUMAN antibody SMN1 antibody SMN2 antibody SMNT antibody Survival motor neuron protein antibody Survival of motor neuron 1; telomeric antibody T-BCD541 antibody
- Application:
- Immunohistochemistry
- translate.label.attr.clone:
- SMN1/1596
- Clonality:
- Monoclonal
- Extra Details:
- 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.
- Host:
- Mouse
- Immunogen:
- Recombinant full-length human SMN1 protein
- Isotype:
- IgG1
- Molecular Weight:
- 39kDa
- Research Area:
- Neuroscience; Signal Transduction
- Shipping Conditions:
- Blue Ice
- Storage Conditions:
- Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous. No MSDS required.
- Supplier:
- NeoBiotechnologies
- Type:
- Antibodies: Monoclonal Antibody
- Manufacturer's Data Sheet:https://www.neobiotechnologies.com/download-datasheet/?PID=139425
