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Rabbit Anti-CRLF1/BF594 Conjugated antibody (bs-8663R-BF594)
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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-8663R-BF594
英文名稱1 Rabbit Anti-CRLF1/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的細(xì)胞因子受體樣因子1抗體
別    名 CISS 1; CISS; CISS1; CLF 1; CLF; CLF1; CRLF 1; Cytokine like factor 1; Cytokine receptor like factor 1; cytokine receptor-like factor 1; NR 6; NR6; ZcytoR 5; ZcytoR5; CRLF1_HUMAN.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)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)  細(xì)胞膜受體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng)
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 43kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CRLF1 (201-300aa)
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:
CRLF1 is a 422 amino acid secreted protein that interacts with cells expressing ciliary neurotrophic factor receptors. A cytokine receptor subunit belonging to the type I cytokine receptor family and type 3 subfamily, CRLF1 is thought to play a role in fetal nervous system development and immunity. CRLF1 is highly expressed in stomach, placenta, heart, ovary, thyroid, bone marrow, appendix, lymph node, spleen, thymus and fetal lung, and promotes neuronal cell survival. CRLF3 is a 442 amino acid protein that contains one fibronectin type-III domain. CRLF3 is expressed in lesion actinic keratosis (AK) and skin and squamous cell carcinoma (SCC), and is thought to negatively regulate the G0/G1 phase of the cell cycle.

Function:
CRLF1 (Cytokine receptor-like factor 1) is a cytokine receptor subunit. It forms a heteromeric complex with cardiotrophin-like cytokine (CLC) and the CRLF1/CLC complex is a ligand for the ciliary neurotrophic factor receptor (CNTFR). Mutations in CRLF1 are responsible for both Crisponi and cold-induced sweating syndromes.

Subunit:
Forms covalently linked di- and tetramers. Forms a heteromeric complex with cardiotrophin-like cytokine (CLC); the CRLF1/CLC complex is a ligand for the ciliary neurotrophic factor receptor (CNTFR).

Subcellular Location:
Secreted

Tissue Specificity:
Highest levels of expression observed in spleen, thymus, lymph node, appendix, bone marrow, stomach, placenta, heart, thyroid and ovary. Strongly expressed also in fetal lung.

DISEASE:
Defects in CRLF1 are the cause of cold-induced sweating syndrome type 1 (CISS1) [MIM:272430]. Cold-induced sweating syndrome (CISS) is an autosomal recessive disorder characterized by profuse sweating induced by cool surroundings (temperatures of 7 to 18 degrees Celsius). Additional abnormalities include a high-arched palate, nasal voice, depressed nasal bridge, inability to fully extend the elbows and kyphoscoliosis.
Defects in CRLF1 are the cause of Crisponi syndrome (CRISPS) [MIM:601378]. Crisponi syndrome is a rare autosomal recessive disorder characterized by congenital muscular contractions of facial muscles, with trismus in response to stimuli, dysmorphic features, bilateral camptodactyly, major feeding and respiratory difficulties, and access of hyperthermia leading to death in the first months of life.

Similarity:
Belongs to the type I cytokine receptor family. Type 3 subfamily.
Contains 2 fibronectin type-III domains.
Contains 1 Ig-like C2-type (immunoglobulin-like) domain.

Database links:
UniProtKB/Swiss-Prot: O75462.1

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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