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RabbitAnti-ATIC/PURH Rabbit pAb  antibody (bs-12544R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-12544R
英文名称 ATIC/PURH Rabbit pAb
中文名称 AICAR甲酰基转移酶抗体
别    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  
研究领域 肿瘤  细胞生物  信号转导  新陈代谢  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,  (predicted: Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, )
产品应用 WB=1:500-2000 ELISA=1:5000-10000 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 65 kDa
检测分子量
细胞定位 细胞浆 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ATIC/AICAR transformylase: 351-450/592 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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



产品图片
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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