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    首頁>>免疫學>>一抗>>指甲髕骨綜合征相關蛋白NPS1抗體
    指甲髕骨綜合征相關蛋白NPS1抗體
    • 產品貨號:
      BN40328R
    • 中文名稱:
      指甲髕骨綜合征相關蛋白NPS1抗體
    • 英文名稱:
      Rabbit anti-LMX1b Polyclonal antibody
    • 品牌:
      Biorigin
    • 貨號

      產品規格

      售價

      備注

    • BN40328R-100ul

      100ul

      ¥2360.00

      交叉反應:Mouse,Rat(predicted:Human,Dog,Pig,Cow,Sheep) 推薦應用:WB,IHC-P,IHC-F,ICC,IF,ELISA

    • BN40328R-200ul

      200ul

      ¥3490.00

      交叉反應:Mouse,Rat(predicted:Human,Dog,Pig,Cow,Sheep) 推薦應用:WB,IHC-P,IHC-F,ICC,IF,ELISA

    產品描述

    英文名稱LMX1b
    中文名稱指甲髕骨綜合征相關蛋白NPS1抗體
    別    名LIM homeo box transcription factor 1 beta; LIM homeobox transcription factor 1 beta; LIM homeobox transcription factor 1-beta; LIM-homeobox protein 1.2; LIM/homeobox protein 1.2; LIM/homeobox protein LMX1B; LMX 1.2; LMX-1.2; LMX1.2; LMX1B; LMX1B_HUMAN; NPS 1; NPS1.  
    研究領域細胞生物  發育生物學  神經生物學  表觀遺傳學  
    抗體來源Rabbit
    克隆類型Polyclonal
    交叉反應Mouse, Rat,  (predicted: Human, Dog, Pig, Cow, Sheep, )
    產品應用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蠟切片需做抗原修復)
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量42kDa
    細胞定位細胞核 
    性    狀Liquid
    濃    度1mg/ml
    免 疫 原KLH conjugated synthetic peptide derived from human LMX1b/NPS1:111-210/379 
    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
    產品介紹This gene encodes a member of LIM-homeodomain family of proteins containing two N-terminal zinc-binding LIM domains, 1 homeodomain, and a C-terminal glutamine-rich domain. It functions as a transcription factor, and is essential for the normal development of dorsal limb structures, the glomerular basement membrane, the anterior segment of the eye, and dopaminergic and serotonergic neurons. Mutations in this gene are associated with nail-patella syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2010]

    Function:
    Essential for the specification of dorsal limb fate at both the zeugopodal and autopodal levels.

    Subcellular Location:
    Nucleus.

    Tissue Specificity:
    Expressed in most tissues. Highest levels in testis, thyroid, duodenum, skeletal muscle, and pancreatic islets.

    DISEASE:
    Defects in LMX1B are the cause of nail-patella syndrome (NPS) [MIM:161200]; also known as onychoosteodysplasia. NPS is a disease that cause abnormal skeletal patterning and renal dysplasia.

    Similarity:
    Contains 1 homeobox DNA-binding domain.
    Contains 2 LIM zinc-binding domains.

    SWISS:
    O60663

    Gene ID:
    4010

    Database links:

    Entrez Gene: 4010 Human

    Entrez Gene: 16917 Mouse

    Entrez Gene: 114501 Rat

    GenBank: U77457.1 Human

    Omim: 602575 Human

    SwissProt: O60663 Human

    SwissProt: O88609 Mouse

    Unigene: 129133 Human

    Unigene: 39825 Mouse

    Unigene: 92364 Rat



    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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