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Anti -Bloom Syndrome (BLM, BS, RECQ2, RECQL2)
Anti -Bloom Syndrome (BLM, BS, RECQ2, RECQL2)
Pricing
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| Clone |
Host |
Grade |
Applications |
| Monoclonal |
Mouse |
Affinity Purified |
B IH |
|
| The Bloom's syndrome (BS) gene, BLM, plays an important role in the maintenance of genomic stability in somatic cells. The BLM protein is a 1417 amino acid peptide with homology to the RecQ helicases, a subfamily of DExH box-containing DNA and RNA helicases. The BLM protein has similarity to 2 other proteins that are members of the subfamily, namely the gene product encoded by RECQL2, also called the Werner syndrome gene (WRN), and the product of the yeast gene SGS1. These proteins may interact with topoisomerases, have 42 to 44% amino acid identity across the conserved helicase motifs, are of similar length and contain highly negatively charged N-terminal regions and highly positively charged C-terminal regions. | | | The BLM protein is located in the nucleus of normal human cells in the nuclear domain 10 (ND10) or promyelocytic leukemia nuclear (PML) bodies. These structures are punctate deposits of proteins disrupted upon viral infection and in certain human malignancies. BLM was found primarily in ND10 except during S phase, when it colocalized with the WRN product in the nucleolus. The BLM protein is likely to be part of a DNA surveillance mechanism operating during S phase-BLM was found to be part of the BASC (BRCA1-associated genome surveillance) complex, which may serve as a sensor of abnormal DNA structures and/or as a regulator of the postreplication repair process. | | | Bloom syndrome cells show marked genomic instability; in particular, hyperrecombination between sister chromatids and homologous chromosomes-SCE (sister chromatid exchanges). In vitro BLM selectively binds Holliday junctions formed during genetic recombination and acts on recombination intermediates containing a Holliday junction to promote ATP-dependent branch migration. BLM may disrupt potentially recombinogenic molecules that arise at sites of stalled replication forks. | | | Catalog # | B2163-01C | | Applications | Suitable for use in Western Blot and Immunohistochemistry (frozen). Other applications not tested. | | Recommended Dilution | Optimal dilutions to be determined by the researcher. | | Positive Control | HeLa nuclear lysate | | Storage and Stability | May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot Store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer. | | CAS Number | n/a | | Clone Type | Monoclonal | | Isotype | IgG1 | | Clone No | 8J345 | | Host | Mouse | | Source | Human | | Concentration | ~0.7mg/ml | | Form | Supplied as a liquid in PBS, pH 7.2. | | Purity | Purified by Protein A affinity chromatography. | | Immunogen | Recombinant full length protein (Human). | | Specificity | Recognizes human BLM protein. | | | Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological. |
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