SMN1 antibody (AA 31-100) (Alexa Fluor 488)

Catalog No. ABIN1391998

Product Details anti-SMN1 Antibody (Alexa Fluor 488)

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

Binding Specificity: AA 31-100

Reactivity: Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This SMN1 antibody is conjugated to Alexa Fluor 488

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Cross-Reactivity: Mouse

Predicted Reactivity: Human,Rat,Dog,Cow,Pig,Rabbit

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human Gemin 1

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

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

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for SMN1

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

Alternative Name: Gemin 1/SMA (SMN1 Products)

Synonyms: SMN2 antibody, Smn antibody, SMN antibody, BCD541 antibody, GEMIN1 antibody, SMA antibody, SMA1 antibody, SMA2 antibody, SMA3 antibody, SMA4 antibody, SMA@ antibody, SMNT antibody, T-BCD541 antibody, TDRD16A antibody, AI849087 antibody, Gemin1 antibody, SMN1 antibody, QtsA-10002 antibody, fa12d01 antibody, smn antibody, wu:fa12d01 antibody, survival motor neuron protein antibody, survival of motor neuron 1, telomeric antibody, survival motor neuron 1 antibody, survival of motor neuron 2, centromeric antibody, survival motor neuron protein-like antibody, survival motor neuron antibody, LOC461829 antibody, Smn1 antibody, SMN1 antibody, SMN2 antibody, LOC100348318 antibody, SMN antibody, LOC100713418 antibody, LOC100065744 antibody, LOC102176643 antibody, smn1 antibody

Background:

Synonyms: Component of gems 1, Gemin 1, Gemin-1, Gemin1, SMA 1, SMA 2, SMA 3, SMA 4, SMA, SMA1, SMA2, SMA3, SMA4, SMN 1, SMN, SMN-1, SMN_HUMAN, SMN1, SMN2, SMNT, Survival motor neuron protein, Survival of motor neuron 1 telomeric, survival of motor neuron 1, Survival of motor neuron 1, telomeric, T-BCD541, BCD541, SMN_HUMAN.

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.

Pathways: Ribonucleoprotein Complex Subunit Organization