SMN1/2 Antibody / Survival of Motor Neuron 1/2

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Catalog number: R32249
Price: 348 €
Supplier: MBS Polyclonals
Product name: SMN1/2 Antibody / Survival of Motor Neuron 1/2
Quantity: 0.12 ml
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Category: Antibody
Concentration: 2ml sterile DI water, 5mg/ml if reconstituted with 0
Form: Antigen affinity purified
Conjugation: Unconjugated
Clone: Polyclonal antibody
Recognised antigen: SMN1/2 / Survival of Motor Neuron 1/2
Host animal: Rabbit (Oryctolagus cuniculus)
Clonality: Polyclonal (rabbit origin)
Species reactivity: Due to limited knowledge and inability to test the antibody against all known species, Mouse (Mus musculus), Rat , we cannot guarantee that no other cross reactivity can occur, Human (Homo sapiens)
Tested applications: IHC-P, WB
Recommended dilutions: 1-0, 5-1ug/ml, 5ug/ml, IHC (Paraffin): 0, Western blot: 0
Notes: Optimal dilution of the SMN1/2 antibody should be determined by the researcher
Intented use: This SMN1/2 antibodyis to be used only for research purposes and not for diagnostics
Uniprot #: Q16637
Purity: Antigen affinity
Description: 2a, 2b, However, It is thought that gene conversion events may involve the two genes, Multiple transcript variants encoding distinct isoforms have been described, Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, The centromeric copy may be a modifier of disease caused by mutation in the telomeric copy, The critical sequence difference between the two genes is a single nucleotide in exon 7, The protein encoded by this gene localizes to both the cytoplasm and the nucleus, The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region, The telomeric and centromeric copies of this gene are nearly identical and encode the same protein, This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions, This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, Within the nucleus, and 3-8, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, are associated with spinal muscular atrophy, leading to varying copy numbers of each gene, mutations in the centromeric copy do not lead to disease, mutations in this gene, such as hnRNP U protein and the small nucleolar RNA binding protein, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs), the telomeric copy, which is thought to be an exon splice enhancer, The Survival of Motor Neuron gene is part of a 500 kb inverted duplication on chromosome 5q13
Immunogen: Amino acids RRGTGQSDDSDIWDDTALIKAYDKAVASFKH of human SMN1/2 were used as the immunogen for the SMN1/2 antibody
Storage: Avoid repeated freezing and thawing, Celcius or lower, Cycles of freezing and thawing can denaturate the peptide chains of the antibodies and reduce their sensitivity and/or change their affinity, Prepare aliqotes in such a manner so that freeze-thaw cycles are minimized, aliquot and store at -20 deg, the SMN1/2 antibody may be kept for up to one month refrigerated at +4 degrees C, After reconstitution, For long-term
Properties: C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by NJS poly they should be stored frozen at - 24°
Gene target: SMN1/2 / Survival of Motor Neuron 1/2
Short name: SMN1/2 Antibody / Survival of Motor Neuron 1/2
Technique: antibodies against human proteins, antibodies for, Antibody
Alternative name: SMN1/2 (Antibody to) / Survival on Motor Neuron 1/2
Alternative technique: antibodies
Identity: 11117
Gene: SMN1 | More about : SMN1
Long gene name: survival of motor neuron 1, telomeric
Synonyms gene: SMA@ SMA
Synonyms gene name: Kugelberg-Welander disease) , spinal muscular atrophy (Werdnig-Hoffmann disease
Synonyms: BCD541 SMNT SMA1 SMA2 SMA3 GEMIN1 TDRD16A
Synonyms name: gemin-1 tudor domain containing 16A
Locus: 5q13, 2
Discovery year: 1996-12-12
GenBank acession: U18423
Entrez gene record: 6606
Pubmed identfication: 7813012
RefSeq identity: NM_000344
Classification: Tudor domain containing
Havana BLAST/BLAT: OTTHUMG00000099361
Locus Specific Databases: SMN1 - Spinal Muscular Atrophy (SMA) Leiden Muscular Dystrophy pages ALSOD, the Amyotrophic Lateral Sclerosis Online Genetic Database LRG_676

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