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Rabbit Anti-REEP1/BF350 Conjugated antibody (bs-11752R-BF350)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11752R-BF350
英文名稱 Rabbit Anti-REEP1/BF350 Conjugated antibody
中文名稱 BF350標記的受體輔助蛋白1抗體
別    名 C2orf23; Chromosome 2 open reading frame 23; FLJ13110; Receptor accessory protein 1; Receptor expression-enhancing protein 1; Reep1; REEP1_HUMAN; SPG31.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  神經生物學  細胞膜受體  G蛋白偶聯受體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,  (predicted: Human, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Zebrafish, Sheep, )
產品應用 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 22kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human REEP1 (53-150aa)
亞    型 IgG
純化方法 affinity purified by Protein A
儲 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.
產品介紹 background:
Transport of G protein-coupled receptors (GPCRs) to the cell surface membrane is critical for receptor-ligand recognition. Mammalian GPCR odorant receptors (ORs), when heterologously expressed in cells, are poorly expressed on the cell surface. REEP1 (receptor expression-enhancing protein 1), is a 201 amino acid multi-pass mitochondrion membrane protein that belongs to the DP1 family. REEP1 interacts with odorant receptor proteins and may enhance the cell surface expression of odorant receptors. Mutations in the REEP1 gene are the third most common cause of hereditary spastic paraplegia (HSP) after spastin and atlastin gene mutations. Mutations in the REEP1 gene also cause spastic paraplegia autosomal dominant type 31, a neurodegenerative disorder. The REEP1 gene is conserved in chimpanzee, dog, cow, mouse, rat, chicken, zebrafish, A.thaliana and rice, and maps to human chromosome 2p11.2.

Function:
May enhance the cell surface expression of odorant receptors.

Subunit:
Interacts with SPAST and ATL1; it preferentiallyinteracts with SPAST isoform 1. Interacts (via C-terminus) withmicrotubules. Interacts with odorant receptor proteins

Subcellular Location:
Membrane. Mitochondrion membrane; Multi-passmembrane protein. Endoplasmic reticulum.

Post-translational modifications:
Belongs to the DP1 family.

DISEASE:
Defects in REEP1 are the cause of spastic paraplegia autosomal dominant type 31 (SPG31) [MIM:610250]. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body.

Similarity:
Belongs to the DP1 family.

Database links:

Entrez Gene: 65055 Human

Entrez Gene: 52250 Mouse

Entrez Gene: 362384 Rat

Omim: 609139 Human

SwissProt: Q9H902 Human

SwissProt: Q8BGH4 Mouse

SwissProt: D4A193 Rat

Unigene: 368884 Human

Unigene: 146332 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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