SMN1 antibody (full length)

Catalog No. ABIN7879102

The Mouse Monoclonal anti-SMN1 antibody (Clone SMN1-1596) (ABIN7879102) specifically detects SMN1 in IHC (p). The antibody is reactive with Human samples.

Quick Overview for SMN1 antibody (full length) (ABIN7879102)

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

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: This SMN1 antibody is un-conjugated

Application: Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Clone: SMN1-1596

Product Details anti-SMN1 Antibody

Binding Specificity: full length

Purpose: SMN1 Antibody / Survival of Motor Neuron

Purification: Protein G affinity

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

Isotype: IgG1, kappa

Application Details

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

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 0.2 mg/mL

Buffer: 0.2 mg/mL in 1X PBS with 0.1 mg/mL BSA (US sourced), 0.05 % sodium azide

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Aliquot the SMN1 antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Target Details for SMN1

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

Alternative Name: SMN1

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.

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization