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Gemin 1/SMA Polyclonal Antibody, HRP Conjugated

Applications

  • WB
  • ELISA
  • IHC-P
  • IHC-F

Reactivity

  • Mouse

Predicted Reactivity

  • Human
  • Rat
  • Dog
  • Cow
  • Pig
  • Rabbit
Overview
Catalog # bs-11561R-HRP
Product Name Gemin 1/SMA Polyclonal Antibody, HRP Conjugated
Applications WB, ELISA, IHC-P, IHC-F
Reactivity Mouse
Predicted Reactivity Human, Rat, Dog, Cow, Pig, Rabbit
Specifications
Conjugation HRP
Host Rabbit
Source KLH conjugated synthetic peptide derived from human Gemin 1
Immunogen Range 31-100/294
Clonality Polyclonal
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Condition Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Target
Subcellular location Cytoplasm, Nucleus
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.
Application Dilution
WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500