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Rabbit Anti-RNF59/MID1/Cy3 Conjugated antibody (bs-9380R-Cy3)
訂購熱線:400-901-9800
訂購郵箱:sales@73327.net
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@73327.net
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-9380R-Cy3
英文名稱1 Rabbit Anti-RNF59/MID1/Cy3 Conjugated antibody
中文名稱 Cy3標記的環(huán)指蛋白59抗體
別    名 BBBG 1; BBBG1; Finger on X and Y mouse homolog of antibody; FXY; GBBB 1; GBBB1; MID 1; MID-1; Mid1; Midin; Midline 1 (Opitz/BBB syndrome); Midline 1; Midline 1 ring finger; Midline 1 RING finger protein; Midline-1; Midline1; OGS 1; OGS1; OS antibody; OSX; Putative transcription factor XPRF; RING finger protein 59; RNF 59; RNF59; TRI18; TRI18_HUMAN; TRIM 18; TRIM18; Tripartite motif containing protein 18; Tripartite motif protein TRIM18; Tripartite motif-containing protein 18; XPRF; Zinc finger X and Y antibody; ZNFXY.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 細胞生物  免疫學(xué)  鋅指蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Horse, Sheep, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 75kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MID1/Midline-1/RNF59
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(chǎn)品介紹 background:
Midline-1 (Tripartite motif-containing protein 18, Putative transcription factor XPRF, RING finger protein 59) is a 667 amino acid protein encoded by the human gene MID1. Midline-1 belongs to the TRIM/RBCC family and contains two B box-type zinc fingers, one B30.2/SPRY domain, one COS domain, one fibronectin type-III domain and one RING-type zinc finger. Midline-1 is believed to have E3 ubiquitin ligase activity which targets the catalytic subunit of protein phosphatase 2 for degradation. It is a cytoplasmic protein found as a homodimer or heterodimer with Midline-2. It also interacts with IGBP1 (Lymphocyte signaling protein A4). Defects in MID1 are the cause of Opitz syndrome type I (OS-I). OS-I is an X-linked recessive disorder characterized by hypertelorism, genital-urinary defects such as hypospadias in males and splayed labia in females, lip-palate-laryngotracheal clefts, imperforate anus, developmental delay and congenital heart defects. OS-I mutations produce proteins with a decreased affinity for microtubules.

Function:
Has E3 ubiquitin ligase activity towards IGBP1, promoting its monoubiquitination, which results in deprotection of the catalytic subunit of protein phosphatase PP2A, and its subsequent degradation by polyubiquitination.

Subunit:
Homodimer or heterodimer with MID2. Interacts with IGBP1.

Subcellular Location:
Cytoplasm.

Tissue Specificity:
In the fetus, highest expression found in kidney, followed by brain and lung. Expressed at low levels in fetal liver. In the adult, most abundant in heart, placenta and brain.

Post-translational modifications:
Phosphorylated on serine and threonine residues.

DISEASE:
Defects in MID1 are the cause of Opitz GBBB syndrome 1 (OGS1) [MIM:300000]. A congenital midline malformation syndrome characterized by hypertelorism, genital-urinary defects such as hypospadias in males and splayed labia in females, lip-palate-laryngotracheal clefts, imperforate anus, developmental delay and congenital heart defects. Note=MID1 mutations produce proteins with a decreased affinity for microtubules.

Similarity:
Belongs to the TRIM/RBCC family.
Contains 2 B box-type zinc fingers.
Contains 1 B30.2/SPRY domain.
Contains 1 COS domain.
Contains 1 fibronectin type-III domain. [SIMILARITY] Contains 1 RING-type zinc finger.

Database links:

Entrez Gene: 4281 Human

Entrez Gene: 17318 Mouse

Entrez Gene: 54252 Rat

Omim: 300552 Human

SwissProt: O15344 Human

SwissProt: O70583 Mouse

SwissProt: P82458 Rat

Unigene: 27695 Human

Unigene: 689953 Human

Unigene: 34441 Mouse

Unigene: 444905 Mouse

Unigene: 460870 Mouse

Unigene: 15169 Rat



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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