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CRLF1 Rabbit pAb (bs-8663R)  
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產品編號 bs-8663R
英文名稱 CRLF1 Rabbit pAb
中文名稱 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.  
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 Rat (predicted: Human,Mouse,Pig,Sheep,Cow,Chicken,Dog)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 43 kDa
檢測分子量
細胞定位 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CRLF1: 201-300/422 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
O75462

Gene ID:
9244

Database links:

Entrez Gene: 9244 Human

Entrez Gene: 12931 Mouse

Entrez Gene: 290655 Rat

Omim: 604237 Human

SwissProt: O75462 Human

SwissProt: Q9JM58 Mouse

Unigene: 114948 Human

Unigene: 335584 Mouse

Unigene: 38637 Rat



產品圖片
Paraformaldehyde-fixed, paraffin embedded (rat heart tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (CRLF1) Polyclonal Antibody, Unconjugated (bs-8663R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (rat skeletal muscle); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (CRLF1) Polyclonal Antibody, Unconjugated (bs-8663R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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