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Argininosuccinic aciduria belongs to a class of genetic diseases called urea cycle disorders. The urea cycle is a sequence of reactions in the cells of the liver. It processes excess nitrogen, generated when protein is used by the body, to make a compound called urea that is excreted by the kidneys. [citation needed]
Urea plant using ammonium carbamate briquettes, Fixed Nitrogen Research Laboratory, ca. 1930 Carl Bosch, 1927. The Bosch–Meiser process is an industrial process, which was patented in 1922 [1] and named after its discoverers, the German chemists Carl Bosch and Wilhelm Meiser [2] for the large-scale manufacturing of urea, a valuable nitrogenous chemical.
3, to the relatively nontoxic excretion product urea. [6] This occurs at the cost of four "high-energy" phosphate bonds (3 ATP hydrolyzed to 2 ADP and one AMP). The conversion from ammonia to urea happens in five main steps. The first is needed for ammonia to enter the cycle and the following four are all a part of the cycle itself.
It is converted to the less toxic substance urea prior to excretion in urine by the kidneys. The metabolic pathways that synthesize urea involve reactions that start in the mitochondria and then move into the cytosol. The process is known as the urea cycle, which comprises several enzymes acting in sequence.
NH 3 + H + ⇌ NH + 4. Ammonia (NH 3) diffuses into the blood, circulating to the liver to be neutralized by the urea cycle. (N.b. urea is not the same as uric acid, though both are end products of the purine nucleotide cycle, from ammonia and nucleotides
Sodium phenylbutyrate is taken orally or by nasogastric intubation as a tablet or powder, and tastes very salty and bitter. It treats urea cycle disorders, genetic diseases in which nitrogen waste builds up in the blood plasma as ammonia glutamine (a state called hyperammonemia) due to deficiences in the enzymes carbamoyl phosphate synthetase I, ornithine transcarbamylase, or argininosuccinic ...
Selective catalytic reduction (SCR) means converting nitrogen oxides, also referred to as NO x with the aid of a catalyst into diatomic nitrogen (N 2), and water (H 2 O). A reductant, typically anhydrous ammonia (NH 3), aqueous ammonia (NH 4 OH), or a urea (CO(NH 2) 2) solution, is added to a stream of flue or exhaust gas and is reacted onto a ...
For historical reasons, the lab test measuring urea is known as "blood urea nitrogen" (BUN) in the US. The BUN:Cr ratio is a useful measure in determining the type of azotemia and will be discussed in each section below. A normal BUN:Cr is equal to 15. [3]