Product Details

Purity

Greater than 85% as determined by SDS-PAGE.

Target Names

Smn1

Uniprot No.

P97801

Research Area

Neuroscience

Alternative Names

Smn1; Smn; Survival motor neuron protein

Species

Mus musculus (Mouse)

Source

Mammalian cell

Expression Region

1-288aa

Target Protein Sequence

MAMGSGGAGSEQEDTVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFKHALKNGDICETPDKPKGTARRKPAKKNKSQKKNATTPLKQWKVGDKCSAVWSEDGCIYPATITSIDFKRETCVVVYTGYGNREEQNLSDLLSPTCEVANSTEQNTQENESQVSTDDSEHSSRSLRSKAHSKSKAAPWTSFLPPPPPMPGSGLGPGKPGLKFNGPPPPPPLPPPPFLPCWMPPFPSGPPIIPPPPPISPDCLDDTDALGSMLISWYMSGYHTGYYMGFRQNKKEGKCSHTN
Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.

Mol. Weight

35.3kDa

Protein Length

Full Length

Tag Info

N-terminal 10xHis-tagged and C-terminal Myc-tagged

Form

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.

Buffer

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
Note: If you have any special requirement for the glycerol content, please remark when you place the order.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Reconstitution

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Troubleshooting and FAQs

Protein FAQs

Storage Condition

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.

Shelf Life

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.

Lead Time

3-7 business days

Notes

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.

Target Background

Function

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).

Gene References into Functions

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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Subcellular Location

Nucleus, gem. Nucleus, Cajal body. Cytoplasm. Cytoplasmic granule. Perikaryon. Cell projection, neuron projection. Cell projection, axon. Cytoplasm, myofibril, sarcomere, Z line.

Protein Families

SMN family

Tissue Specificity

Expressed in motor neurons.

Database Links

KEGG: mmu:20595

STRING: 10090.ENSMUSP00000022147

UniGene: Mm.2025

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Code	CSB-MP021838MO
MSDS	MSDS
Size	$500
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Image	(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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Recombinant Mouse Survival motor neuron protein (Smn1)

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