BLM (1319-1335) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	ELISA, IHC
Recommend Dilution	ELISA: 1/1000. Immunohistochemistry on Paraffin Sections: 10 µg/ml.
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Immunogen	Synthetic peptide from human BLM, aa 1319-1335
Specificity	Recognises Bloom's Syndrome Protein (BLM) at aa 1319-1335.
Formulation	Phosphate Buffered Saline PBS containing 0.09% Sodium Azide as preservative State: Purified State: Liquid purified IgG fraction
Concentration	lot specific
Purification	Protein G Chromatography
Conjugation	Unconjugated
Storage Condition	Store the antibody undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Dilute only prior to immediate use. Avoid cycles of freezing and thawing.
Gene Name	Bloom syndrome RecQ like helicase
Database Link	Entrez Gene 641 Human UniProt ID P54132
Background	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 Werner syndrome gene product, WRN, 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.
Synonyms	Bloom syndrome protein, RecQ protein-like 3, DNA helicase, RecQ-like type 2, RECQ2, RECQL3
Reference Data

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