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Rabbit Anti-CRLF1/Gold Conjugated antibody (bs-8663R-Gold)
訂購熱線:400-901-9800
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-8663R-Gold
英文名稱1 Rabbit Anti-CRLF1/Gold Conjugated antibody
中文名稱 膠體金標記的細胞因子受體樣因子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)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 細胞生物  免疫學  細胞膜受體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Sheep, )
產(chǎn)品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
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 0.4mg/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 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
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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