AICAR甲酰基转移酶抗体
规格：1mg/1ml
英文名： ATIC/PURH
别名： AICAR transformylase; 5 aminoimidazole 4 carboxamide 1 beta D ribonucleotide transformylase/inosinicase; 5 aminoimidazole 4 carboxamide ribonucleotide formyltransferase; 5 aminoimidazole 4 carboxamide
分子量： 65kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ATIC/AIC
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
细胞定位：
AICAR甲?；泼缚固?/strong>产品介绍：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.AICAR甲酰基转移酶抗体 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 andAICAR甲?；泼缚固?/strong> 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.
AICAR甲?；泼缚固?/strong>产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 信号转导 新陈代谢 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid