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指甲髌骨综合征相关蛋白NPS1抗体
  • 产品货号:
    BN40328R
  • 中文名称:
    指甲髌骨综合征相关蛋白NPS1抗体
  • 英文名称:
    Rabbit anti-LMX1b Polyclonal antibody
  • 货号

    产品规格

    售价

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  • BN40328R-100ul

    100ul

    ¥2360.00

    交叉反应:Mouse,Rat(predicted:Human,Dog,Pig,Cow,Sheep) 推荐应用:WB,IHC-P,IHC-F,ICC,IF,ELISA

  • BN40328R-200ul

    200ul

    ¥3490.00

    交叉反应:Mouse,Rat(predicted:Human,Dog,Pig,Cow,Sheep) 推荐应用:WB,IHC-P,IHC-F,ICC,IF,ELISA

英文名称LMX1b
中文名称指甲髌骨综合征相关蛋白NPS1抗体
别    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.  
研究领域细胞生物  发育生物学  神经生物学  表观遗传学  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Mouse, Rat,  (predicted: Human, Dog, Pig, Cow, Sheep, )
产品应用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 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量42kDa
细胞定位细胞核 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human LMX1b/NPS1:111-210/379 
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
产品介绍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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