| Long name: |
Biotin Conjugated, SMN1 Polyclonal Antibody |
| Category: |
Conjugated Primary Antibodies |
| Conjugation: |
Biotinylated |
| Host Organism: |
Rabbit (Oryctolagus cuniculus) |
| Target Antigen: |
SMN1 |
| Specificity: |
100% sequence similarity to SMN-delta7, SMN-delta5, SMN-delta57, This is a highly specific antibody against SMN1 |
| Modification: |
Unmodified |
| Modification site: |
None |
| Clonality: |
Polyclonal |
| Clone: |
Polyclonal antibody |
| Concentration: |
1ug per 1ul |
| Immunogen_range: |
60-95/294 |
| Source: |
KLH conjugated synthetic peptide derived from human SMN1 |
| Gene ID number: |
6606 |
| Tested applications: |
IHC-P, WB |
| Recommended dilutions: |
IHC-P(1:100-500), WB(1:100-1000) |
| Crossreactivity: |
Mouse, Rat, Human |
| Crossreactive species details: |
not every possible cross-reactivity is known, Due to limited amount of testing and knowledge |
| Antigen background: |
2a, 2b, Four transcript variants encoding distinct isoforms have been described, It is thought that gene conversion events may involve the two genes, Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, The critical sequence difference between the two genes is a single nucleotide in exon 7, The full length 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 gene may be a modifier of disease caused by mutation in the telomeric copy, This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, While mutations in the telomeric copy are associated with spinal muscular atrophy, Within the nucleus, and 3-8, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, do not lead to disease, leading to varying copy numbers of each gene, mutations in this gene, such as hnRNP U protein and the small nucleolar RNA binding protein, the centromeric copy, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs), which is thought to be an exon splice enhancer, SMN1 is part of a 500 kb inverted duplication on chromosome 5q13 |
| Purification method: |
This antibody was purified via Protein A |
| Storage conditions: |
50% glycerol and 0, Store refrigerated at 2 to 8 degrees Celcius for up to 1 year, 09% sodium azide, Keep the antibody in an aqueous buffered solution containing 1% BSA |
| Synonyms: |
BCD541 SMNT SMA1 SMA2 SMA3 GEMIN1 TDRD16A |
| Properties: |
Avidin and Streptavidin bind to the small biotin and are couple to HRP or AP for ELISA, Biotin conjugates can be detected by horseradish peroxidase, To break the streptavidin Biotin bond we suggest to use a 6 molar guanidine HCl solution with acidity of pH 1, alkaline phosphatase substrates or anti biotin conjugated antibodies, 6, C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24° |
| Gene target: |
SMN1 Conjugated |
| Short name: |
Conjugated, SMN1 Antibody |
| Technique: |
antibodies against human proteins, antibodies for, antibody Conjugates, Antibody |
| Label: |
Biotin |
| Alternative name: |
biotinilated coupled, SMN1 (Antibody to) |
| 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 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 |