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Rabbit Anti-DIF14/LMBR1/BF350 Conjugated antibody (bs-9563R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-9563R-BF350
英文名稱 Rabbit Anti-DIF14/LMBR1/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的分化相關(guān)基因14抗體
別    名 ACHP; C7orf2; DIF 14; DIF14; Differentiation related gene 14; Differentiation related gene 14 protein; Differentiation-related gene 14 protein; FLJ11665; Limb region 1 homolog (mouse); Limb region 1 homolog; Limb region 1 protein; Limb region 1 protein homolog; LMBR 1; LMBR1; LMBR1_HUMAN; PPD 2; PPD2; TPT.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  發(fā)育生物學(xué)  細(xì)胞分化  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 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 55kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LMBR1/DIF14
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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(chǎn)品介紹 background:
LMBR1 is a 490 amino acid multi-pass membrane protein that is widely expressed with strongest expression in heart and pancreas. Belonging to the LIMR family, LMBR1 shares 95% sequence identity with the mouse protein and may play crucial role in the evolution of limb and skeletal system. LMBR1 is critical for expression of sonic hedgehog (Shh) in the developing posterior limb bud mesenchyme. Mutations in the gene encoding LMBR1 is the cause of several rare conditions such as acheiropody (ACHP) and syndactyly type 4 (SDYT4). ACHP is an autosomal recessive inherited disorder characterized by bilateral congenital amputations of the hands and feet. LMBR1L (limb region 1 protein homolog-like), also known as LIMR (Lipocalin-1-interacting membrane receptor), is a 489 amino acid multi-pass membrane protein that is thought to act as a receptor for Lipocalin-1 and may also assist in its endocytosis.

Function:
Putative membrane receptor.

Subcellular Location:
Membrane.

Tissue Specificity:
Widely expressed with strongest expression in heart and pancreas.

DISEASE:
Defects in LMBR1 are associated with preaxial polydactyly type 2 (PPD2); also known as polydactyly of triphalangeal thumb. Polydactyly consists of duplication of the distal phalanx. The thumb in PPD2 is usually opposable and possesses a normal metacarpal. The mutations do not change the normal expression of LMBR1, but alter the expression of SHH by disrupting a long-range, cis-regulatory element of that gene.

Similarity:
Belongs to the LIMR family.

Database links:

Entrez Gene: 64327 Human

Entrez Gene: 56873 Mouse

Entrez Gene: 362295 Rat

Omim: 605522 Human

SwissProt: Q8WVP7 Human

SwissProt: Q9JIT0 Mouse

Unigene: 209989 Human



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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