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Rabbit Anti-ATIC/PURH/BF488 Conjugated antibody (bs-12544R-BF488)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-12544R-BF488
英文名稱1 Rabbit Anti-ATIC/PURH/BF488 Conjugated antibody
中文名稱 BF488標記的AICAR甲?;D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-aminoimidazole-4-carboxamide ribonucleotide formyltransferase; AICAR; AICAR formyltransferase/IMP cyclohydrolase bifunctional enzyme; AICARFT; AICARFT/IMPCHASE; ATIC; Bifunctional purine biosynthesis protein PURH; FLJ93545; IMP cyclohydrolase; IMP synthase; IMP synthetase; IMPCHASE; Inosinicase; OK/SW-cl.86; Phosphoribosylaminoimidazolecarboxamide formyltransferase; Phosphoribosylaminoimidazolecarboxamide formyltransferase/IMP cyclohydrolase; PUR9_HUMAN; PURH.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
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 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, )
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 65kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ATIC/AICAR transformylase
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:
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.

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



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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