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货号： bs-8663R-Bio 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-8663R-Bio
- 英文名称
- Anti-CRLF1/Biotin
- 中文名称
- 生物素标记的细胞因子受体样因子1抗体
- 别 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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 免疫学 细胞膜受体
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Sheep,
- 产品应用
- 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
- 43kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human CRLF1 (201-300aa)
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- 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.
- 产品介绍
- 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.