AICAR, AICARFT, AICARFT/IMPCHASE, PURH, 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase,The bifunctional purine biosynthesis protein PURH contains phosphoribosylaminoimidazole carboxamide formyltransferase, also designated AICAR transformylase, IMP cyclohydrolase or Inosinicase. AICAR plays an important role in purine biosynthesis, specifically in the production of nucleotides and IMP. Defects in ATIC, the gene encoding for this protein, can cause AICArebosuria, also designated AICA-ribosiduria, an inborn error in purine biosynthesis that is neurologically cataclysmic. Individuals with AICA-rebosuria accumulate AICA-ribotide, also designated ZMP, and its derivatives in erythrocytes and fibroblasts and 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.
Aufreinigung
Protein A/G Chromatography
Immunogen
Hybridoma produced by the fusion of splenocytes from BALB/c mice immunized with a synthetic peptide derived from the human ATIC protein and mouse myeloma Ag8563 cells. Sequence common in frog, fruit fly, rat and mouse.
Antibody can be used for Western blotting (1-2 μg/mL) and immunohistochemistry on formalin-fixed, paraffin-embedded tissues (1-5 μg/mL). Optimal concentration should be evaluated by serial dilutions.
Beschränkungen
Nur für Forschungszwecke einsetzbar
Buffer
Provided as solution in phosphate buffered saline with 0.08 % sodium azide
Konservierungsmittel
Sodium azide
Vorsichtsmaßnahmen
This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Lagerung
-20 °C
Informationen zur Lagerung
Product should be stored at -20°C. Aliquot to avoid freeze/thaw cycles