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  4. Recombinant Mouse Survival motor neuron protein (Smn1)

Recombinant Mouse Survival motor neuron protein (Smn1)

In Stock
  • 中文名称：
    小鼠Smn1重组蛋白
  • 货号：
    CSB-MP021838MO
  • 规格：
    ￥3000
  • 图片：
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他：

产品详情

  • 纯度：
    Greater than 85% as determined by SDS-PAGE.
  • 基因名：
  • Uniprot No.：
  • 别名：
    Smn1; Smn; Survival motor neuron protein
  • 种属：
    Mus musculus(Mouse)
  • 蛋白长度：
    Full Length
  • 来源：
    Mammalian cell
  • 分子量：
    35.3kDa
  • 表达区域：
    1-288aa
  • 氨基酸序列
    MAMGSGGAGSEQEDTVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFKHALKNGDICETPDKPKGTARRKPAKKNKSQKKNATTPLKQWKVGDKCSAVWSEDGCIYPATITSIDFKRETCVVVYTGYGNREEQNLSDLLSPTCEVANSTEQNTQENESQVSTDDSEHSSRSLRSKAHSKSKAAPWTSFLPPPPPMPGSGLGPGKPGLKFNGPPPPPPLPPPPFLPCWMPPFPSGPPIIPPPPPISPDCLDDTDALGSMLISWYMSGYHTGYYMGFRQNKKEGKCSHTN
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal 10xHis-tagged and C-terminal Myc-tagged
  • 产品提供形式：
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 缓冲液：
    Tris-based buffer,50% glycerol
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    3-7 business days
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA：
    Please contact us to get it.

产品评价

靶点详情

  • 功能：
    The SMN complex catalyzes the assembly of small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome, and thereby plays an important role in the splicing of cellular pre-mRNAs. Most spliceosomal snRNPs contain a common set of Sm proteins SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE, SNRPF and SNRPG that assemble in a heptameric protein ring on the Sm site of the small nuclear RNA to form the core snRNP (Sm core). In the cytosol, the Sm proteins SNRPD1, SNRPD2, SNRPE, SNRPF and SNRPG are trapped in an inactive 6S pICln-Sm complex by the chaperone CLNS1A that controls the assembly of the core snRNP. To assemble core snRNPs, the SMN complex accepts the trapped 5Sm proteins from CLNS1A forming an intermediate. Binding of snRNA inside 5Sm ultimately triggers eviction of the SMN complex, thereby allowing binding of SNRPD3 and SNRPB to complete assembly of the core snRNP. Within the SMN complex, SMN1 acts as a structural backbone and together with GEMIN2 it gathers the Sm complex subunits. Ensures the correct splicing of U12 intron-containing genes that may be important for normal motor and proprioceptive neurons development. Also required for resolving RNA-DNA hybrids created by RNA polymerase II, that form R-loop in transcription terminal regions, an important step in proper transcription termination. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs).
  • 基因功能参考文献：
    1. Survival Motor Neuron (SMN) protein is required for normal mouse liver development PMID: 27698380
    2. Widespread intron retention, particularly of minor U12 introns, in the spinal cord of mice 30 d after SMA induction. PMID: 28270613
    3. A proteomic profile of embryonic stem cells with low smn protein revealed thematic changes consistent with the developmental dysfunction seen in the pathophysiological development of patients with spinal muscular atrophy. Pathways associated with mRNA spicing, protein translation, post-translational modification and perhaps most striking, mitochondrial function and specifically mitochondrial dysfunction were highlighted. PMID: 27145767
    4. Loganin is capable of increas-ing the SMN protein level under SMN-deficient conditions both inin vitro and in vivo models of spinal muscular atrophy via Akt/mTOR pathway. PMID: 27241020
    5. miR-431 expression was highly increased, and a number of its putative mRNA targets were significantly downregulated in motor neurons after SMN loss. Further, we found that miR-431 regulates motor neuron neurite length by targeting several molecules previously identified to play a role in motor neuron axon outgrowth, including chondrolectin PMID: 27005422
    6. To determine the dependence of oligodendrocyte (OL)on the Smn protein(SMN1), we utilized the Smn-/-;SMN2 (severe) mouse model. Our data suggest that despite the multi-functionality and ubiquitous expression of the Smn protein, it does not play a key role in myelination of the CNS, at least in the context of spinal muscular atrophy pathogenesis. PMID: 28069797
    7. our studies show that this G-motif represents a novel and essential determinant for axonal localization of the Anxa2 mRNA mediated by the SMN complex. PMID: 28258160
    8. A long non-coding RNA (lncRNA) that arises from the antisense strand of SMN, SMN-AS1, is enriched in neurons and transcriptionally represses SMN expression by recruiting the epigenetic Polycomb repressive complex-2. PMID: 28017471
    9. SMN1 expression restoration is curative in a spinal muscular atrophy model mice. PMID: 27907033
    10. Survival motor neuron 1, and survival motor neuron 2, depletion results in increased alternative splicing events. PMID: 27736905
    11. these results demonstrate that SMN deficiency impacts spleen development and suggests a potential role for immunological development in Spinal muscular atrophy. PMID: 28062667
    12. Itch monoubiquitinates SMN and monoubiquitination of SMN plays an important role in regulating its cellular localization. PMID: 26908624
    13. muscle does not appear to require high levels of SMN above what is produced by two copies of SMN2 PMID: 26276812
    14. Findings demonstrate that high expression of SMN in the motor neuron is both necessary and sufficient for proper function of the motor unit. In addition, SMN high expression in neurons and glia has a major impact on survival. PMID: 26206889
    15. This study identifies pathways related to the function of Smn and associated with differential motor unit vulnerability, thus presenting a number of exciting targets for future therapeutic development. PMID: 26374403
    16. Smn complex deficiency caused constipation, delayed gastric emptying, slow intestinal transit and reduced colonic motility. PMID: 25859009
    17. Primary cell culture and two different SMA model mice to demonstrate that reduced levels of Smn lead to a profound disruption in the expression of myogenic genes. PMID: 24691550
    18. Results suggest that SMN plays a role in the maintenance of pluripotent embryonic stem cells and neuronal differentiation in mice. PMID: 24633826
    19. AAV9-mediated SMN gene therapy elicits cure for spinal muscular atrophy. PMID: 25358252
    20. Data show that changes in U12 introns-dependent splicing become apparent after prolonged/extensive survival motor neuron proteins SMN depletion. PMID: 25692239
    21. This work both reveals a new autoregulatory pathway governing SMN expression, and identifies a new mechanism through which SMN can modulate specific mRNA expression via Gemin5. PMID: 25911097
    22. SMN is involved in the axonal translocation of hnRNP R and hnRNP R-bound RNA/protein complexes. PMID: 25338097
    23. Findings are consistent with a role for SMN in myotube formation through effects on muscle differentiation and cell motility. PMID: 24760765
    24. Data indicate that Smn deficiency affects the subcellular transcriptome in both the somatodendritic and axonal regions of motoneurons impairing transcripts with selected functions. PMID: 25246652
    25. Findings suggest that carriers of SMN1 mutations and/or deletions may be at an increased risk of developing pancreatic and glucose metabolism defects. PMID: 24497575
    26. Data support a role for SMN in the regulation of mRNA localization and axonal transport through its interaction with mRNA-binding proteins such as IMP1 PMID: 23897586
    27. It improves neuromuscular function and motor neuron survival in mutant SOD1 mice. PMID: 24210254
    28. SMN1 is essential for U7 biogenesis and histone mRNA processing. PMID: 24332368
    29. sregulation of UBA1 and subsequent ubiquitination pathways led to beta-catenin accumulation in a model of mutant SMN spinal muscular atrophy. PMID: 24590288
    30. Restoration of SMN to Emx-1 expressing cortical neurons is not sufficient to provide benefit to a severe mouse model of Spinal Muscular Atrophy. PMID: 23512182
    31. enhanced Survival Motoneuron protein has a role in neuromuscular junction maturation PMID: 24463453
    32. a new mechanism for regulating SMN levels and provides new insight into the roles of U1A in 3' processing of mRNAs. PMID: 24362020
    33. Study shows that neuronal aggregates formed by mutant FUS protein may aberrantly sequester survival motor neuron protein and concomitantly cause a reduction of SMN levels in the axon, leading to axonal defects. PMID: 23681068
    34. Smn protein reduction causes an increment of the autophagosome number as well as of the autophagy-related proteins Beclin1 and LC3-II. PMID: 23788043
    35. Low SMN levels might result in localization deficiencies of mRNAs required for axonogenesis. PMID: 24152552
    36. SAHA significantly increases SMN levels and also increased vascular density in SMA mice (P<0.05), suggesting that the vascular defect in SMA mice is amenable to SAHA treatment. PMID: 23583590
    37. Overexpressing the SMN protein in mutant SOD1 mice, a model of familial ALS, alleviates this phenomenon, most likely in a cell-autonomous manner, and significantly mitigates the loss of motor neurons in the spinal cord and in culture dishes. PMID: 22581780
    38. Expressing SMN pan-neuronally in a mouse model of severe spinal muscular atrophy results in a four-fold increase in survival. PMID: 23029491
    39. Data suggest a critical role of aurvival of motor neuron SMN1 and SMN2 proteins in the intrinsic regulation of muscle differentiation and suggest that abnormal muscle development contributes to the manifestation of spinal muscular atrophy (SMA) symptoms. PMID: 22705478
    40. SMN plays distinct roles in muscle, neuromuscular junctions, and motor neuron somal synapses PMID: 22723710
    41. This study identified a critical threshold of Smn that dictates onset of SMA in the intermediate Smn(2B/-) mouse model. PMID: 22071333
    42. Hyper-phosphorylation of profilin2a is the molecular link between SMN and the ROCK pathway repressing neurite outgrowth in neuronal cells. PMID: 21920940
    43. SMN is essential for the normal postnatal maturation of motor nerve terminals. PMID: 22022549
    44. comparison of systemic versus CNS restoration of SMN in a severe mouse model PMID: 21979052
    45. This study demonistrated that the spinal muscular atrophy mouse model, SMADelta7, displays altered axonal transport without global neurofilament alterations PMID: 21681521
    46. Prolactin increases SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway PMID: 21785216
    47. even in severe SMA, timely reinstatement of the SMN protein may halt the progression of the disease PMID: 21785219
    48. Data show that SMN and HuD form a complex in spinal motor axons, and that both interact with cpg15 mRNA in neurons. PMID: 21652774
    49. Involvement of SMN in formation of stress granules may play an important role in cell survival. PMID: 21234798
    50. Mouse survival motor neuron alleles that mimic SMN2 splicing and are inducible rescue embryonic lethality early in development but not late [SMN2] PMID: 21249120

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  • 亚细胞定位：
    Nucleus, gem. Nucleus, Cajal body. Cytoplasm. Cytoplasmic granule. Perikaryon. Cell projection, neuron projection. Cell projection, axon. Cytoplasm, myofibril, sarcomere, Z line.
  • 蛋白家族：
    SMN family
  • 组织特异性：
    Expressed in motor neurons.
  • 数据库链接：

    KEGG: mmu:20595

    STRING: 10090.ENSMUSP00000022147

    UniGene: Mm.2025