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Urea Cycle
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Biochemistry
Professional
01/09/2012

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Term
Pepsinogen
Definition
important proteases

converted to active pepsin in gastric juice by autocatalysis of pepsin itself

pepsin hydrolyzes those peptide bonds of ingested proteins involving aromatic amino acids

cleavage pt on amion terminal side in stomach
Term
trypsinogen
Definition
converted to active by enteropeptidase

acts on lys and arginine peptide bonds at the carboyxl group

in the small intestine
Term
Chymotrpsinogen
Definition
converted by trypsin

breaks bonds involving Tyr, Phe, Trp at the carboxyl-group
Term
Carboxypeptidase A
Definition
inactive = procarboxypeptidase

cleaves c-terminal residues with aromatic or aliphatic side chains at the peptide bond adjacet to the C-terminal end of polypeptides (Tyr, Trp, Phe or Gly, Ala, Val, Leu, Ile)

in small intestine
Term
Carboxypeptidase B
Definition
cleaves basic amino acids (Lys, Arg and Ornithine) from the C-terminal position of polypeptides

in the small intestine
Term
Aminopeptidase
Definition
cleaves amino terminal residues (except proline) from the peptide bond adjacent to N-terminal end of polypeptides

in the small intestine
Term
Gastrin
Definition
stomach polypeptide hormones

stimulates gastric HCl secretion
Term
Secretin
Definition
small intestine polypeptide hormone

stimulates bicarbonate secretion to neutralize the gastric HCl and increase the pH to about 7
Term
Cholecystokinin
Definition
small intestine polypeptide horomone

promotes secretion of several pancreatic enzymes with optimal activity at pH 7 to 8
Term
First Step in Most Amino Acid Degradation
Definition
transfer the amino group to alpha-ketoglutarate to form L-glutamate
Term
Amino Acids degraded to Pyruvate
Definition
Threonine
Glycine
Serine
Cystein
Tryptophan
Alanine
Term
Glycine Degradation major pathway
Definition
yields ammonia and carbon dioxide and it requires a tetrahydrofolate reaction, which brings the oxidative cleavage of glycine into Coz and NH4
Term
Degratdation of Cysteine
Definition
can form pyruvate, taurine, PAPS
Term
Amino Acids Degraded to Acetyl-CoA (7)
Definition
Tryptophan, Lysine, Phenylalanine, Tyrosine, Leucine, Isoleucine, and Threonine
Term
Degradation of Tryptophan
Definition
either to pyruvate, kynurenine, or indolepyruvate

the kynurenine leads to produciton of 2 Acetyl-CoA
Term
Amino Acids converted to alpha-ketoglutarate
Definition
Arginine, Histidine, Glutamate, Glutamine, and Proline
Term
Amino Acids Converted to Succinyl-CoA
Definition
Methionine, Isoleucine, Threonine, Valine
Term
Amino Acids Converted to oxaloacetate
Definition
Asparagine, Aspartic Acid
Term
Amino Acids converted to Fumarate
Definition
Tyrosine, Phenylalanine
Term
Ketone Bodies
Definition
Acetoacetate, D-Beta-hydroxybutyrate, acetone

water-soluble compounds that are produced as by-products when fatty acids are broken down for energy in the liver and kidney
Term
Glucogenic
Definition
amino acids degraded to pyruvate, alpha-ketoglutarate, succinyl-CoA, fummarate and / or oxaloacetate can be converted to glucose and glycogen

all except leucine + lysine
Term
Ketogenic
Definition
amino acids degraded entirely or in part to acetoacetyl-coA and or acetyl-CoA, can yield ketone bodies in the liver

Leucine + lysine and those that are both
Term
Glucogenic and ketogenic
Definition
amino acids that have both activities

F, Y, W, T, I
Term
Where does urea synthesis take place
Definition
primarily in the tissues of the liver
Term
Pre-Step of Urea Cycle
Definition
1st amino group enters the urea cycle in a form of free amonia by the deamination of glutamate inside the mitochondria of the liver cells
Term
1st Major reaction of Urea Cycle
Definition
Atp dependent reaction COz + NH4+ form carbamoyl phosphate through the enzyme carbamoyl phosphate synthetase I

two terminal phosphate groups of ATP are used to form molecule of carbamoyl phosphate
Term
2nd Major Reaction of Urea Cycle
Definition
carbamoyl phosphate donates its carbamoyl group to ornithine to form citrulline and release phosphate

catalyzed by ornithine transcarbamoylase

citrulline leaves mitochondria and passes into the cytosol of liver cells
Term
3rd Major Reaction of Urea Cycle
Definition
Aspartate donates amine group to citrulline to form arginiosuccinate condensation reaction

catalyzed by argininouccinate synthetase (mg) dependent
Term
4th Major Reaction of Urea Cycle
Definition
Argininosuccinate will be cleaved to Arginine and Fumarate by argininosuccinase

fumarate returns to the pool of CAC intermediates
Term
5th Major Reaction in the Urea Cycle
Definition
Arginase cleaves arginine to yield urea and prnithine

ornithine is regenerated and can enter the mitochondria again to intitiate another round of the urea cycle
Term
6 Major Urea Cycle disorders
Definition
Each is referred to by the initials of the missing enzyme

CPS, NAGS, OTC, AS, AL/ASA, AG
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