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Mouse Monoclonal SMN1 antibody [5H1] (STJ98390)
Supplier: St John’s Laboratory Ltd.
Recommended applications: WB, IHC, IF, ELISA
Recommended dilution: WB 1:500-1:2000; IHC 1:200-1:1000; IF 1:200-1:1000; ELISA 1:10000
Recommended protocols: check protocols
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Check alternative names for the antibodyExpand
SMN1 antibody, SMN antibody, SMNT; antibody, SMN2 antibody, SMNC antibody,|BCD541 antibody|Component of gems 1 antibody|Gemin 1 antibody|Gemin-1 antibody|OTTHUMP00000125198 antibody|OTTHUMP00000223567 antibody|OTTHUMP00000223568 antibody|OTTHUMP00000224066 antibody|OTTHUMP00000226924 antibody|SMA 1 antibody|SMA 2 antibody|SMA 3 antibody|SMA 4 antibody|SMA antibody|SMA@ antibody|SMA1 antibody|SMA2 antibody|SMA3 antibody|SMA4 antibody|SMN antibody|SMN_HUMAN antibody|SMN1 antibody|SMN2 antibody|SMNT antibody|Survival motor neuron protein antibody|Survival of motor neuron 1, telomeric antibody|T-BCD541 antibody|Anti-Gemin 1 antibody [2B1] (ab5831)
SCBT cat No: To be updated
SMN1 Monoclonal Antibody
|Catalogue No.|| |
SMN1 Monoclonal Antibody detects endogenous levels of SMN1 protein.
Purified recombinant fragment of human SMN1 expressed in E Coli
WB, IHC, IF, ELISA
|Recommended dilution|| |
WB 1:500-1:2000; IHC 1:200-1:1000; IF 1:200-1:1000; ELISA 1:10000
SMN1 Antibody was tube-contained. Ascitic fluid containing 0.03% sodium azide.
SMN1 Antibody was purified using affinity purification.
-20 Celsius degree. Avoid repeated freeze/thaw cycles.
|Alternative antibody names|| |
Survival motor neuron protein antibody, Component of gems 1 antibody, Gemin-1 antibody
|Protein names|| |
Survival motor neuron protein , Component of gems 1 , Gemin-1
|Protein function|| |
The SMN complex plays a catalyst role in the assembly of small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome. 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. 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. Dissociation by the SMN complex of CLNS1A from the trapped Sm proteins and their transfer to an SMN-Sm complex triggers the assembly of core snRNPs and their transport to the nucleus. 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).
|Protein tissue specificity|| |
Expressed in a wide variety of tissues. Expressed at high levels in brain, kidney and liver, moderate levels in skeletal and cardiac muscle, and low levels in fibroblasts and lymphocytes. Also seen at high levels in spinal cord. Present in osteoclasts and mononuclear cells (at protein level).
|Involvement in disease|| |
Spinal muscular atrophy 1 (SMA1) [MIM:253300]: A form of spinal muscular atrophy, a group of neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Autosomal recessive forms are classified according to the age of onset, the maximum muscular activity achieved, and survivorship. The severity of the disease is mainly determined by the copy number of SMN2, a copy gene which predominantly produces exon 7-skipped transcripts and only low amount of full-length transcripts that encode for a protein identical to SMN1. Only about 4% of SMA patients bear one SMN1 copy with an intragenic mutation. SMA1 is a severe form, with onset before 6 months of age. SMA1 patients never achieve the ability to sit. . Note: The disease is caused by mutations affecting the gene represented in this entry.; Spinal muscular atrophy 2 (SMA2) [MIM:253550]: An autosomal recessive form of spinal muscular atrophy, a neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. It has intermediate severity, with onset between 6 and 18 months. Patients do not reach the motor milestone of standing, and survive into adulthood. . Note: The disease is caused by mutations affecting the gene represented in this entry.; Spinal muscular atrophy 3 (SMA3) [MIM:253400]: An autosomal recessive form of spinal muscular atrophy, a neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Onset is after 18 months. Patients develop ability to stand and walk and survive into adulthood. . Note: The disease is caused by mutations affecting the gene represented in this entry.; Spinal muscular atrophy 4 (SMA4) [MIM:271150]: An autosomal recessive form of spinal muscular atrophy, a neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Onset is in adulthood, disease progression is slow, and patients can stand and walk. . Note: The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
The Tudor domain mediates association with dimethylarginines, which are common in snRNP proteins. / Belongs to the SMN family. / Contains 1 Tudor domain.
|Protein cellular localization|| |
Cytoplasm / Nucleus > gem / Nucleus > Cajal body / Cytoplasmic granule / Cytoplasm > myofibril > sarcomere > Z line
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St John’s Laboratory Ltd.
|Product type|| |
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