Recombinant Mouse SMN/Gemin 1 Protein (Tagged)

Collections: Other recombinant proteins, Recombinant proteins

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Product Overview

Host Species	Mouse
Accession	P97801
Synonym	BCD541 Component of gems 1 Gemin 1 Gemin-1 OTTHUMP00000125198 OTTHUMP00000223567 OTTHUMP00000223568 OTTHUMP00000224066 OTTHUMP00000226924 SMA SMA 1 SMA 2 SMA 3 SMA 4 SMA@ SMA1 SMA2 SMA3 SMA4 SMN SMN_HUMAN SMN1 SMN2 SMNT Survival motor neuron protein Survival of motor neuron 1, telomeric T-BCD541
Description	Recombinant Mouse SMN/Gemin 1 Protein (Tagged) was expressed in Mammalian. It is a Full length protein
Source	Mammalian
AA Sequence	MAMGSGGAGSEQEDTVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFKHA LKNGDICETPDKPKGTARRKPAKKNKSQKKNATTPLKQWKVGDKCSAVWS EDGCIYPATITSIDFKRETCVVVYTGYGNREEQNLSDLLSPTCEVANSTE QNTQENESQVSTDDSEHSSRSLRSKAHSKSKAAPWTSFLPPPPPMPGSGL GPGKPGLKFNGPPPPPPLPPPPFLPCWMPPFPSGPPIIPPPPPISPDCLD DTDALGSMLISWYMSGYHTGYYMGFRQNKKEGKCSHTN
Purity	>85% SDS-PAGE.
Endotoxin	< 1.0 EU per μg of the protein as determined by the LAL method
Formulation	Liquid Solution
Stability	The recombinant protein samples are stable for up to 12 months at -80°C
Reconstitution	See related COA
Unit Definition	For Research Use Only
Storage Buffer	Shipped at 4°C. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

Target Details

Target 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).
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
Database References	KEGG: mmu:20595 STRING: 10090.ENSMUSP00000022147 UniGene: Mm.2025
Tissue Specificity	Expressed in motor neurons.

Gene Functions References

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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Our protein products do not contain carrier protein or other additives (such as bovine serum albumin (BSA), human serum albumin (HSA) and sucrose, etc., and when lyophilized with the solution with the lowest salt content, they often cannot form A white grid structure, but a small amount of protein is deposited in the tube during the freeze-drying process, forming a thin or invisible transparent protein layer.

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