Recombinant Mouse Survival motor neuron protein (Smn1)
In Stock-
中文名稱:小鼠Smn1重組蛋白
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貨號(hào):CSB-MP021838MO
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規(guī)格:¥3000
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圖片:
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其他:
產(chǎn)品詳情
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純度:Greater than 85% as determined by SDS-PAGE.
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基因名:
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Uniprot No.:
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別名:Smn1; Smn; Survival motor neuron protein
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種屬:Mus musculus(Mouse)
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蛋白長(zhǎng)度:Full Length
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來(lái)源:Mammalian cell
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分子量:35.3kDa
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表達(dá)區(qū)域:1-288aa
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氨基酸序列MAMGSGGAGSEQEDTVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFKHALKNGDICETPDKPKGTARRKPAKKNKSQKKNATTPLKQWKVGDKCSAVWSEDGCIYPATITSIDFKRETCVVVYTGYGNREEQNLSDLLSPTCEVANSTEQNTQENESQVSTDDSEHSSRSLRSKAHSKSKAAPWTSFLPPPPPMPGSGLGPGKPGLKFNGPPPPPPLPPPPFLPCWMPPFPSGPPIIPPPPPISPDCLDDTDALGSMLISWYMSGYHTGYYMGFRQNKKEGKCSHTN
Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request. -
蛋白標(biāo)簽:N-terminal 10xHis-tagged and C-terminal Myc-tagged
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產(chǎn)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. -
緩沖液:Tris-based buffer,50% glycerol
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儲(chǔ)存條件:Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
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保質(zhì)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. -
貨期:3-7 business days
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注意事項(xiàng):Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
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Datasheet & COA:Please contact us to get it.
相關(guān)產(chǎn)品
靶點(diǎn)詳情
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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).
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基因功能參考文獻(xiàn):
- Survival Motor Neuron (SMN) protein is required for normal mouse liver development PMID: 27698380
- Widespread intron retention, particularly of minor U12 introns, in the spinal cord of mice 30 d after SMA induction. PMID: 28270613
- 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
- 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
- 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
- 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
- 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
- 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
- SMN1 expression restoration is curative in a spinal muscular atrophy model mice. PMID: 27907033
- Survival motor neuron 1, and survival motor neuron 2, depletion results in increased alternative splicing events. PMID: 27736905
- these results demonstrate that SMN deficiency impacts spleen development and suggests a potential role for immunological development in Spinal muscular atrophy. PMID: 28062667
- Itch monoubiquitinates SMN and monoubiquitination of SMN plays an important role in regulating its cellular localization. PMID: 26908624
- muscle does not appear to require high levels of SMN above what is produced by two copies of SMN2 PMID: 26276812
- 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
- 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
- Smn complex deficiency caused constipation, delayed gastric emptying, slow intestinal transit and reduced colonic motility. PMID: 25859009
- 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
- Results suggest that SMN plays a role in the maintenance of pluripotent embryonic stem cells and neuronal differentiation in mice. PMID: 24633826
- AAV9-mediated SMN gene therapy elicits cure for spinal muscular atrophy. PMID: 25358252
- Data show that changes in U12 introns-dependent splicing become apparent after prolonged/extensive survival motor neuron proteins SMN depletion. PMID: 25692239
- 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
- SMN is involved in the axonal translocation of hnRNP R and hnRNP R-bound RNA/protein complexes. PMID: 25338097
- Findings are consistent with a role for SMN in myotube formation through effects on muscle differentiation and cell motility. PMID: 24760765
- 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
- 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
- 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
- It improves neuromuscular function and motor neuron survival in mutant SOD1 mice. PMID: 24210254
- SMN1 is essential for U7 biogenesis and histone mRNA processing. PMID: 24332368
- sregulation of UBA1 and subsequent ubiquitination pathways led to beta-catenin accumulation in a model of mutant SMN spinal muscular atrophy. PMID: 24590288
- 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
- enhanced Survival Motoneuron protein has a role in neuromuscular junction maturation PMID: 24463453
- a new mechanism for regulating SMN levels and provides new insight into the roles of U1A in 3' processing of mRNAs. PMID: 24362020
- 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
- Smn protein reduction causes an increment of the autophagosome number as well as of the autophagy-related proteins Beclin1 and LC3-II. PMID: 23788043
- Low SMN levels might result in localization deficiencies of mRNAs required for axonogenesis. PMID: 24152552
- 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
- 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
- Expressing SMN pan-neuronally in a mouse model of severe spinal muscular atrophy results in a four-fold increase in survival. PMID: 23029491
- 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
- SMN plays distinct roles in muscle, neuromuscular junctions, and motor neuron somal synapses PMID: 22723710
- This study identified a critical threshold of Smn that dictates onset of SMA in the intermediate Smn(2B/-) mouse model. PMID: 22071333
- Hyper-phosphorylation of profilin2a is the molecular link between SMN and the ROCK pathway repressing neurite outgrowth in neuronal cells. PMID: 21920940
- SMN is essential for the normal postnatal maturation of motor nerve terminals. PMID: 22022549
- comparison of systemic versus CNS restoration of SMN in a severe mouse model PMID: 21979052
- This study demonistrated that the spinal muscular atrophy mouse model, SMADelta7, displays altered axonal transport without global neurofilament alterations PMID: 21681521
- Prolactin increases SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway PMID: 21785216
- even in severe SMA, timely reinstatement of the SMN protein may halt the progression of the disease PMID: 21785219
- Data show that SMN and HuD form a complex in spinal motor axons, and that both interact with cpg15 mRNA in neurons. PMID: 21652774
- Involvement of SMN in formation of stress granules may play an important role in cell survival. PMID: 21234798
- 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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亞細(xì)胞定位:Nucleus, gem. Nucleus, Cajal body. Cytoplasm. Cytoplasmic granule. Perikaryon. Cell projection, neuron projection. Cell projection, axon. Cytoplasm, myofibril, sarcomere, Z line.
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蛋白家族:SMN family
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組織特異性:Expressed in motor neurons.
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