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首頁>>免疫學(xué)>>一抗>>Bloom綜合征相關(guān)蛋白抗體
Bloom綜合征相關(guān)蛋白抗體
  • 產(chǎn)品貨號:
    BN40711R
  • 中文名稱:
    Bloom綜合征相關(guān)蛋白抗體
  • 英文名稱:
    Rabbit anti-BLM Polyclonal antibody
  • 品牌:
    Biorigin
  • 貨號

    產(chǎn)品規(guī)格

    售價

    備注

  • BN40711R-100ul

    100ul

    ¥2360.00

    交叉反應(yīng):Mouse,Rat(predicted:Human,Pig,Horse,Rabbit) 推薦應(yīng)用:IHC-P

  • BN40711R-200ul

    200ul

    ¥3490.00

    交叉反應(yīng):Mouse,Rat(predicted:Human,Pig,Horse,Rabbit) 推薦應(yīng)用:IHC-P

產(chǎn)品描述

英文名稱BLM
中文名稱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)  表觀遺傳學(xué)  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Mouse, Rat,  (predicted: Human, Pig, Horse, Rabbit, )
產(chǎn)品應(yīng)用IHC-P=1:100-500 (石蠟切片需做抗原修復(fù))
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量159kDa
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
儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.



















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