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RNF135 Rabbit pAb (bs-11753R)  
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產品編號 bs-11753R
英文名稱 RNF135 Rabbit pAb
中文名稱 環指蛋白135抗體
別    名 L13; MGC13061; ring finger protein 135; RN135_HUMAN .  
研究領域 神經生物學  轉錄調節因子  表觀遺傳學  環指蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Cow,Dog)
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 48 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human RNF135: 288-360/432 
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 The RING-type zinc finger motif is present in a number of viral and eukaryotic proteins and is made of a conserved cysteine-rich domain that is able to bind two zinc atoms. Proteins that contain this conserved domain are generally involved in the ubiquitination pathway of protein degradation. RNF135 (RING finger protein 135), also known as L13, is a 432 amino acid protein that contains one RING-type zinc finger and one SPRY domain. Via its RING-type zinc finger, RNF135 may play a role in transcriptional regulation and protein degradation events. Defects in the gene encoding RNF135 are the cause of RNF135-related overgrowth syndrome which is characterized by learning disabilities, facial dysmorphism and increased weight and height. Multiple isoforms of RNF135 exist due to alternative splicing events.

Function:
The protein encoded by RNF135 contains a RING finger domain, a motif present in a variety of functionally distinct proteins and known to be involved in protein-protein and protein-DNA interactions. This gene is located in a chromosomal region known to be frequently deleted in patients with neurofibromatosis.

Subunit:
Interacts with DDX58. Interacts with PCBP2.

Subcellular Location:
Cytoplasmic

Tissue Specificity:
Expressed in skeletal muscle, spleen, kidney,placenta, prostate, stomach, thyroid and tongue. Also weaklyexpressed in heart, thymus, liver and lung.

Post-translational modifications:
Defects in RNF135 are the cause of macrocephalymacrosomia facial dysmorphism syndrome (MMFD) [MIM:614192]. MMFD isan autosomal dominant disorder characterized by the association ofmacrothrombocytopathy and progressive sensorineural hearing losswithout renal dysfunction.

Similarity:
Contains 1 B30.2/SPRY domain.
Contains 1 RING-type zinc finger.

SWISS:
Q8IUD6

Gene ID:
84282

Database links:

Entrez Gene: 84282 Human

Entrez Gene: 71956 Mouse

Omim: 611358 Human

SwissProt: Q8IUD6 Human

SwissProt: Q9CWS1 Mouse

Unigene: 29874 Human

Unigene: 22985 Mouse



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