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    BLM Rabbit pAb (bs-22614R)  
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    產(chǎn)品編號 bs-22614R
    英文名稱(chēng) BLM Rabbit pAb
    中文名稱(chēng) Bloom綜合征相關(guān)蛋白抗體
    別    名 Blooms Syndrome Protein Blm; BLM; BLM_HUMAN; Bloom Syndrome; Bloom syndrome protein; Bloom syndrome RecQ helicase like; BS; DNA Helicase; DNA helicase RecQ like type 2; MGC126616; MGC131618; MGC131620; RECQ 2; RECQ like; RecQ like type 2; RecQ protein like 3; RecQ Protein-like 3; RECQ-2; RECQ-Like; RecQ-like type 2; RECQ2; RECQL 2; RECQL 3; RECQL-2; RECQL-3; RECQL2; RECQL3; type 2.  
    研究領(lǐng)域 細胞生物  發(fā)育生物學(xué)  表觀(guān)遺傳學(xué)  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Mouse,Rat (predicted: Human,Rabbit,Pig,Horse)
    產(chǎn)品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 159 kDa
    檢測分子量
    細胞定位 細胞核 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human BLM: 761-860/1417 
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    產(chǎn)品介紹 Bloom’s syndrome is an autosomal recessive disorder characterized by pre- and post-natal growth deficiencies, sun sensitivity, immunodeficiency and a predisposition to various cancers. The gene responsible for Bloom’s syndrome, BLM, encodes a protein homologous to the RecQ helicase of E. coli and is mutated in most Bloom’s syndrome patients. One characteristic of Bloom’s syndrome is an increased frequency of sister chromatid exchange (SCE). BLM has been shown to unwind G4 DNA, and a failure of this function is thought to be responsible for the increased rate of SCE. BLM is known to be translocated to the nucleus, where its ATPase activity is stimulated by both single- and double-stranded DNA. Mutations in the yeast SGS1, a homolog of BLM, are known to cause mitotic hyperrecombination similiar to that observed in Bloom’s cells.

    Function:
    Participates in DNA replication and repair. Exhibits a magnesium-dependent ATP-dependent DNA-helicase activity that unwinds single- and double-stranded DNA in a 3'-5' direction.

    Subunit:
    Part of the BRCA1-associated genome surveillance complex(BASC), which contains BRCA1, MSH2, MSH6, MLH1, ATM, BLM, PMS2 andthe RAD50-MRE11-NBS1 protein complex. This association could be adynamic process changing throughout the cell cycle and withinsubnuclear domains. Interacts with ubiquitinated FANCD2. Interactswith RMI complex. Interacts directly with RMI1 component of RMIcomplex. Interacts with SUPV3L1.

    Subcellular Location:
    Nucleus.

    Post-translational modifications:
    Phosphorylated in response to DNA damage. Phosphorylation requires the FANCA-FANCC-FANCE-FANCF-FANCG protein complex, as well as the presence of RMI1.

    DISEASE:
    Defects in BLM are the cause of Bloom syndrome (BLM) [MIM:210900]. BLM is an autosomal recessive disorder characterized by proportionate pre- and postnatal growth deficiency, sun-sensitive telangiectatic hypo- and hyperpigmented skin, predisposition to malignancy, and chromosomal instability.

    Similarity:
    Belongs to the helicase family. RecQ subfamily.
    Contains 1 helicase ATP-binding domain.
    Contains 1 helicase C-terminal domain.
    Contains 1 HRDC domain.

    SWISS:
    P54132

    Gene ID:
    641

    Database links:

    Entrez Gene: 641 Human

    Entrez Gene: 12144 Mouse

    Omim: 604610 Human

    SwissProt: P54132 Human

    SwissProt: O88700 Mouse

    Unigene: 725208 Human

    Unigene: 12932 Mouse



    產(chǎn)品圖片
    Paraformaldehyde-fixed, paraffin embedded (mouse kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (BLM ) Polyclonal Antibody, Unconjugated (bs-22614R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
    Paraformaldehyde-fixed, paraffin embedded (mouse intestine); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (BLM ) Polyclonal Antibody, Unconjugated (bs-22614R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
    Paraformaldehyde-fixed, paraffin embedded (mouse testis); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (BLM ) Polyclonal Antibody, Unconjugated (bs-22614R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
    Paraformaldehyde-fixed, paraffin embedded (rat intestine); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (BLM ) Polyclonal Antibody, Unconjugated (bs-22614R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
    Paraformaldehyde-fixed, paraffin embedded (rat kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (BLM ) Polyclonal Antibody, Unconjugated (bs-22614R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
    Paraformaldehyde-fixed, paraffin embedded (mouse pancreas); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (BLM ) Polyclonal Antibody, Unconjugated (bs-22614R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
    Paraformaldehyde-fixed, paraffin embedded (rat pancreas); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (BLM ) Polyclonal Antibody, Unconjugated (bs-22614R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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