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SMN1 蛋白

(Survival of Motor Neuron 1, Telomeric (SMN1))
This gene is part of a 500 kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. However, mutations in this gene, the telomeric copy, are associated with spinal muscular atrophy\; mutations in the centromeric copy do not lead to disease. The centromeric copy may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7, which is thought to be an exon splice enhancer. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Two transcript variants encoding distinct isoforms have been described. [provided by RefSeq, Sep 2008].

10 results :

custom-made SMN1 宿主: 小鼠 宿主: HEK-293 Cells Recombinant > 90 % as determined by Bis-Tris PAGE, anti-tag ELISA, Western Blot and analytical SEC (HPLC)
产品编号 ABIN7563565
 
SMN1 宿主: 人 宿主: 小麦胚 Recombinant ELISA, WB, AP, AA
产品编号 ABIN1320585
 
SMN1 宿主: 人 宿主: 大肠杆菌(E. Coli) Recombinant ELISA, WB, SDS
产品编号 ABIN7579597
 
SMN1 宿主: 人 宿主: 大肠杆菌(E. Coli) Recombinant > 97 % WB, SDS, Imm, PC
产品编号 ABIN7416892
 
custom-made SMN1 宿主: 小鼠 宿主: Cell-free protein synthesis (CFPS) Recombinant approximately 70-80 % as determined by SDS PAGE, Western Blot and analytical SEC (HPLC). ELISA, WB, SDS
产品编号 ABIN3134732
 
SMN1 宿主: Cow 宿主: 酵母菌 Recombinant > 90 % ELISA
产品编号 ABIN7586168
 
SMN1 宿主: 大鼠 宿主: 酵母菌 Recombinant > 90 % ELISA
产品编号 ABIN7586169
 
SMN1 宿主: 犬 宿主: 酵母菌 Recombinant > 90 % ELISA
产品编号 ABIN1461410
 
SMN1 宿主: Cat 宿主: 酵母菌 Recombinant > 90 % ELISA
产品编号 ABIN1636281
 
SMN1 宿主: Cynomolgus 宿主: 酵母菌 Recombinant > 90 % ELISA
产品编号 ABIN1631650
 
  • 类型 Protein
    • Protein
  • 应用范围
    • ELISA
    • Western Blotting (WB)
    • SDS-PAGE (SDS)
    • Affinity Purification (AP)
    • Antibody Array (AA)
    • Immunogen (Imm)
    • Positive Control (PC)
  • 适用
    • Human
    • Mouse
    • Cat
    • Cow
    • Cynomolgus
    • Dog
    • Rat
  • 图像
  • Carrier free only
  • 抗体来源
    • Yeast
    • Escherichia coli (E. coli)
    • Cell-free protein synthesis (CFPS)
    • HEK-293 Cells
    • Wheat germ
  • 抗原表位
    • AA 1-287
    • AA 1-288
    • AA 2-294
    • AA 1-140
    • AA 1-289
    • AA 1-290
  • 标记
    • His tag
    • GST tag
    • Strep Tag
  • 蛋白类型
    • Recombinant
  • Format
    • Lyophilized
    • Liquid
  • Grade
    • custom-made
  • Supplier
    • CUSABIO
    • antibodies-online

Aliases for SMN1 蛋白

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