ITM1053
ITM1053
- Catalog: ITM1053
- Gene/Protein: ATIC
- Product Description: Immunotag™ IMP cyclohydrolase Monoclonal Antibody
504.0000
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Antibody Specification | |
Datasheet | |
Target Protein | IMP ch |
Clonality | Monoclonal |
Storage/Stability | -20°C/1 year |
Application | WB |
Recommended Dilution | Western Blot: 1/1000 - 1/2000. Not yet tested in other applications. |
Concentration | 1 mg/ml |
Reactive Species | Human,Rb |
Host Species | Mouse |
Immunogen | Purified recombinant human IMP cyclohydrolase protein fragments expressed in Ecoli |
Specificity | IMP cyclohydrolase Monoclonal Antibody detects endogenous levels of IMP cyclohydrolase protein. |
Purification | Affinity purification |
Form | Purified mouse monoclonal in buffer containing 0.1M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.2% sodium azide, 50% glycerol. |
Gene Name | ATIC |
Accession No. | P31939 Q9CWJ9 O35567 |
Alternate Names | ATIC; PURH; OK/SW-cl.86; Bifunctional purine biosynthesis protein PURH |
Description | 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase(ATIC) Homo sapiens This gene encodes a bifunctional protein that catalyzes the last two steps of the de novo purine biosynthetic pathway. The N-terminal domain has phosphoribosylaminoimidazolecarboxamide formyltransferase activity, and the C-terminal domain has IMP cyclohydrolase activity. A mutation in this gene results in AICA-ribosiduria. [provided by RefSeq, Sep 2009], |
Cell Pathway/ Category | Purine metabolism,One carbon pool by folate, |
Protein Expression | Brain,Cajal-Retzius |
Subcellular Localization | mitochondrion,cytosol,cell-cell adherens junction,membrane,extracellular exosome, |
Protein Function | catalytic activity:10-formyltetrahydrofolate + 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide = tetrahydrofolate + 5-formamido-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide.,catalytic activity:IMP + H(2)O = 5-formamido-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide.,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.,domain:The IMP cyclohydrolase activity resides in the N-terminal region.,pathway:Purine metabolism; IMP biosynthesis via de novo pathway; 5-formamido-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide from 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide (10-formyl THF route): step 1/1.,pathway:Purine metabolism; IMP biosynthesis via de novo pathway; IMP from 5-formamido-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide: step 1/1.,similarity:Belongs to the purH family., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |