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ATIC/PURH Rabbit pAb (bs-12544R)  
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產品編號 bs-12544R
英文名稱 ATIC/PURH Rabbit pAb
中文名稱 AICAR甲酰基轉移酶抗體
別    名 AICAR transformylase; 5 aminoimidazole 4 carboxamide 1 beta D ribonucleotide transformylase/inosinicase; 5 aminoimidazole 4 carboxamide ribonucleotide formyltransferase; 5 aminoimidazole 4 carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase; 5  
研究領域 腫瘤  細胞生物  信號轉導  新陳代謝  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human (predicted: Mouse,Rat,Rabbit,Pig,Cow,Dog,Horse)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 65 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ATIC/AICAR transformylase: 351-450/592 
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 bifunctional purine biosynthesis protein ATIC (also designated PURH) contains AICAR transformylase and IMP cyclohydrolase activities. AICAR (5-aminoimidazole-4-carboxamide ribonucleotide) transformylase catalyzes the second to last step in purine biosynthesis, playing an important role in the production of nucleotides and IMP. Defects in the ATIC transformylase gene can cause AICA-rebsuria, also designated AICA-ribosiduria, an inborn error in purine biosynthesis that is neurologically cataclysmic. Individuals with AICA-rebosuria accumulate AICA-riboside, also designated ZMP, and its derivatives in erythrocytes and fibroblasts. Patients also excrete very large amounts of AICA-riboside in the urine. Mental retardation, epilepsy, dysmorphic features and congenital blindness are all symptoms of this disease.

Function:
Bifunctional enzyme that catalyzes 2 steps in purine biosynthesis.

Subunit:
Homodimer.

DISEASE:
Defects in ATIC are the cause of AICA-ribosuria [MIM:608688]; also known as AICA-ribosiduria. AICA-ribosuria is a neurologically devastating inborn error of purine biosynthesis. AICA-ribosuria patients excrete massive amounts of AICA-riboside in the urine and accumulate AICA-ribotide and its derivatives in erythrocytes and fibroblasts. AICA-ribosuria causes profound mental retardation, epilepsy, dysmorphic features and congenital blindness.

Similarity:
Belongs to the purH family.

SWISS:
P31939

Gene ID:
471

Database links:

Entrez Gene: 471 Human

Entrez Gene: 108147 Mouse

Entrez Gene: 81643 Rat

Entrez Gene: 396091 Chicken

Omim: 601731 Human

SwissProt: P31335 Chicken

SwissProt: P31939 Human

SwissProt: Q9CWJ9 Mouse

SwissProt: O35567 Rat

Unigene: 90280 Human

Unigene: 38010 Mouse

Unigene: 15114 Rat



產品圖片
Sample: K562 (Human) Cell Lysate at 30 ug HepG2 (Human) Cell Lysate at 30 ug Primary: Anti- ATIC’PURH (bs-12544R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 65 kD Observed band size: 65 kD
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