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Rabbit Anti-LMX1b/Biotin Conjugated antibody (bs-11780R-Bio)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11780R-Bio
英文名稱 Rabbit Anti-LMX1b/Biotin Conjugated antibody
中文名稱 生物素標記的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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
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=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 42kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LMX1b/NPS1
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 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
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.

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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