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Rabbit Anti-LRRC6/BF350 Conjugated antibody (bs-13613R-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-13613R-BF350
英文名稱 Rabbit Anti-LRRC6/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的富含亮氨酸重復(fù)蛋白6抗體
別    名 Leucine rich repeat containing 6; Leucine rich repeat containing protein 6; Leucine rich testis specific protein; Leucine-rich repeat-containing protein 6; Leucine-rich testis-specific protein; Lrrc6; LRTP; Protein TILB homolog; Testis specific leucine rich repeat protein; Testis-specific leucine-rich repeat protein; TILB_HUMAN; TSLRP.  
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  發(fā)育生物學(xué)  干細(xì)胞  轉(zhuǎn)錄調(diào)節(jié)因子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, 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 54kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LRRC6
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:
The leucine-rich repeat-containing protein 6 (LRRC6), also designated Leucine-rich testis-specific protein (LRTP), is a 466 amino acid protein that contains 3 LRR repeats and plays a role in spermatogenesis. The gene encoding LRRC6 maps to chromosome 8, which encodes approximately 800 genes. Translocation of portions of chromosome 8 with amplifications of the c-Myc gene are found in some leukemias and lymphomas, and typically associated with a poor prognosis. Chromosome 8 is also associated with Trisomy 8, Pfeiffer syndrome, congenital hypothyroidism and Waardenburg syndrome.

Function:
May play a role in dynein arm assembly, hence essential for proper axoneme building for cilia motility.

Subcellular Location:
Cytoplasm. Cell projection > cilium.

Tissue Specificity:
Expressed predominantly in testis and in nasal epithelial cells.

DISEASE:
Defects in LRRC6 are the cause of primary ciliary dyskinesia 19 (CILD19) [MIM:614935]. A disorder characterized by abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit randomization of left-right body asymmetry and situs inversus, due to dysfunction of monocilia at the embryonic node. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.

Similarity:
Belongs to the tilb protein family.
Contains 1 CS domain.
Contains 4 LRR (leucine-rich) repeats.
Contains 1 LRRCT domain.

Database links:

Entrez Gene: 23639 Human

Entrez Gene: 54562 Mouse

Entrez Gene: 299920 Rat

SwissProt: Q86X45 Human

SwissProt: O88978 Mouse

Unigene: 591865 Human

Unigene: 244890 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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