Mouse anti Human ATIC

• Background:

  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.

• Synonyms:

  AICAR, AICARFT, AICARFT/IMPCHASE, PURH, 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase

• UniProt:

  P31939

• Host:

  Mouse

• Species Reactivity:

  Human, Frog, Fruitfly, Rat, Mouse

• Isotype:

  IgG1

• Clone:

  F38 P7 H9

• Conjugation:

  Unconjugated

• Type:

  Monoclonal Antibody

• Applications:

  WB, IHC

• Field of Research:

  Signal Transduction

• Purification Method:

  Protein A/G Chromatography

• Assay Principle:

  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.

• Stability:

  See expiration date on vial

• Concentration:

  See vial for Concentration

• Form:

  Provided as solution in phosphate buffered saline with 0,08% sodium azide

• Precautions:

  This product is intended FOR RESEARCH USE ONLY, and FOR TESTS IN VITRO, not for use in diagnostic or therapeutic procedures involving Humans or animals.

• References & Citations:

  1. Bulock, K.G., et al. (2002). The kinetic mechanism of the Human bifunctional enzyme ATIC (5-amino-4-imidazolecarboxamide ribonucleotide transformylase/inosine 5'-monophosphate cyclohydrolase). A surprising lack of substrate channeling. J. Biol. Chem. 277(25):22168-22174. 2. Marie, S., et al. (2004). AICA-ribosiduria: a novel, neurologically devastating inborn error of purine biosynthesis caused by mutation of ATIC. Am. J. Hum. Genet. 74(6):1276-1281.

• Shipping Conditions:

  Ambient Temperature, freeze upon arrival

• Storage Conditions:

  Product should be stored at -20ºC; Aliquot to avoid freeze/thaw cycles

• Functional Analysis:

  WB

• CAS Number:

  9007-83-4