Carbamyl phosphate synthase

Carbamyl phosphate synthase deficiency p Glnt, Citt, Alat, Lyst... 

Ovalbumin Conalbumin Ovomucoid Lysozyme Vitellogenin apo-VLDL Glucose-6-P-dehydrogenase Oviduct Oviduct (liver) Oviduct Oviduct Liver Liver Uterus Thyroid Hormones Carbamyl phosphate synthase Growth hormone Prolactin ( ) a-Glycerophosphate dehydrogenase Malic enzyme Liver Pituitary Pituitary Liver (mitochondria) Liver... 

The uiea cycle may be considered to be a mitochondrial pathway, as carbamyl phosphate synthase and ornithine transcarbamylase are mitochondrial enzymes however, the enzymes catalyzing subsequent steps of the pathway arc cytosolic-The steps leading to conversion of citrulline to ornithine occur in the cytosol. Hence, the pathway is shared by the mitochondrial and cytosolic compartments. The fumarate produced by the urea cycle is converted to malate by a cytoplasmic form of fumarase. Mittxihondrial fumarase is part of the Krebs cycle. Cytoplasmic malate can enter the mitochondrion by means of a transport system, such as the malate/phosphate exchanger or the ma ate/a-ketoglutaratc exchanger. These transport systems are membrane-bound proteins. 

Fahien, L. A., Kmiotek, E. H., Woldegiorgis, G., Evenson, M., Shrago, E., and Marshall, M. (1985). Regulation of aminotransferase-glutamate dehydrogenase interactions by carbamyl phosphate synthase. I. Mg plus leucine versus citrate and malate. /. Biol. Chem. 260, 6069-6079. 

Father G, et al. Carbamyl phosphate synthase deficiency diagnosed diuing pregnancy in a 41-year-old. J Clin Neurosci. 2006 13(6) 702-6. 

R51. Use of phosphorus-31 and carbon-13 NMR to study enzyme mechanisms Villafranca, J. J. Fed. Proc., Fed. Am. Soc. Exp. Biol. 1984, 11, 2640-2647 (30 references). Chiefly concerned with glutamine synthetase and carbamyl phosphate synthase. 

Humans cannot synthesize biotin and depend on dietary biotin originating in microbial and plant biosynthetic pathways. The route of biosynthesis of biotin was largely elaborated by Rolfe and Eisenberg (1968) working with Escherichia coll. In this pathway, dethiobiotin is formed from pimelyl-CoA (which can be synthesized from oleic acid) and carbamyl phosphate. Sulfur is incorporated into dethiobiotin in a synthase-dependent step, generating biotin (reviewed by Cam-poreale and Zempleni 2006). Figure 10.1 shows the chemical structure of biotin. 

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