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Chapter 18
Amino Acid Oxidation & the Production of Urea
41
Biochemistry
Undergraduate 3
03/14/2016

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Term
gastrin
Definition
the hormone secreted by the gastric mucosa when there is entry of dietary protein into the stomach; it stimulates the secretion of hydrochloric acid by the parietal cells & pepsinogen by the chief cells of the gastric glands
Term
pepsinogen
Definition
an inactive precursor (zymogen) which is converted to active pepsin by an autocatalytic cleavage that occurs only at low pH
Term
secretin
Definition
the hormone secreted as the acidic stomach contents pass into the small intestine & the pH becomes low; released into the blood
Term
cholecystokinin
Definition
the hormone released into the blood due to the arrival of amino acids in the upper part of the intestine; stimulates secretion of several pancreatic enzymes with activity optima at pH 7 to 8
Term
3 zymogens that are synthesized & secreted by the exocrine cells of the pancreas
Definition
1. trypsinogen (becomes trypsin)
2. chymotrypsinogen (becomes chymotrypsin)
3. procarboxypeptidases A & B (become carboxypeptidases A & B)
Term
enteropeptidase
Definition
converts trypsinogen to its active form (trypsin); a proteolytic enzyme secreted by intestinal cells
Term
pancreatic trypsin inhibitor
Definition
a protein which is a specific inhibitor made by the pancreas to protect itself against self-digestion; effectively prevents premature production of active proteolytic enzymes within the pancreatic cells
Term
aminopeptidase
Definition
hydrolyzes successive amino-terminal residues from short peptides
Term
acute pancreatitis
Definition
a disease caused by obstruction of the normal pathway by which pancreatic secretions enter the intestine
Term
amino-transferases (transaminases)
Definition
enzymes which promote the removal of the alpha-amino groups from most L-amino acids once they have reached the liver for the first step of catabolism
Term
transamination reactions
Definition
the alpha-amino group is transferred to the alpha-carbon atom of alpha-ketoglutarate, leaving behind the corresponding alpha-keto acid analog of the amino acid
Term
pyridoxal phosphate (PLP)
Definition
a prosthetic group; the coenzyme form of pyridoxine (vitamin B6)
Term
What is the relationship between oxidative deamination & L-glutamate dehydrogenase?
Definition
in hepatocytes, glutamate is transported from the cytosol into the mitochondria where it undergoes oxidative deamination catalyzed by L-glutamate dehydrogenase
Term
transdeamination
Definition
the combined action of an aminotransferase & glutamate dehydrogenase
Term
glutamine synthetase
Definition
allows for the free ammonia produced in tissues to combine with glutamate to yield glutamine
Term
glucose-alanine cycle
Definition
a pathway in which alanine plays a special roe in transporting amino groups to the liver in a nontoxic form
Term
alanine aminotransferase
Definition
allows for glutamate to transfer its alpha-amino group to pyruvate (a readily available product of muscle glycolysis) as an alternative to glutamate being converted to glutamine for transport to the liver
Term
ammonotelic
Definition
most aquatic species (ex. bony fishes) are ammonotelic; they excrete amino nitrogen as ammonia
Term
ureotelic
Definition
most terrestrial animals are ureotelic; they excrete amino nitrogen in the form of urea
Term
urea cycle
Definition
how the ammonia deposited in the mitochondria of hepatocytes is converted to urea in ureotelic organisms
Term
argininosuccinase
Definition
cleaves argininosuccinate; forms free arginine & fumarate (fumarate is converted to malate before entering the mitochondria to join the pool of citric acid cycle intermediates)
Term
What is the relationship between arginase & urea?
Definition
in the last reaction of the urea cycle, the cytosolic enzyme arginase cleaves arginine to yield urea & ornithine
Term
aspartate-argininosuccinate shunt
Definition
provides metabolic links between the separate pathways by which the amino groups & carbon skeletons of amino acids are processed
Term
What is the relationship between N-acetylglutamate & N-acetylglutamate synthase?
Definition
Carbamoyl phosphate synthetase I is allosterically activated by N-acetylglutamate which is synthesized from acetyl-CoA & glutamate by N-acetylglutamate synthase
Term
essential amino acids
Definition
the half of the 20 common amino acids which humans are incapable of synthesizing; must be provided in the diet
Term
tetrahydrofolate (H4 folate)
Definition
synthesized in bacteria & consists of substituted pterin, p-aminobenzoate, & glutamate moieties
Term
S-Adenosylmethionine (adoMet)
Definition
the preferred cofactor for biological methyl group transfers
Term
methionine adenosyl transferase
Definition
synthesizes adoMet from ATP & methionine
Term
S-adenosylhomocysteine
Definition
yielded by the transfer of the methyl group from S-Adenosylmethionine to an acceptor; is broken down to homocysteine & adenosine
Term
pernicious anemia
Definition
the B12 deficiency disease; a rare disease seen only in individuals who have a defect in intestinal absorption pathways for this vitamin or in strict vegetarians
Term
megaloblastic anemia
Definition
the anemia associated with vitamin B12 deficiency
Term
megaloblasts
Definition
immature precursor cells in the bone marrow whose appearance (along with a decline in the production of mature erythrocytes) is associated with megaloblastic anemia
Term
macrocytes
Definition
abnormally large erythrocytes which gradually replace erythrocytes in the blood in smaller numbers in individuals with megaloblastic anemia
Term
What is the relationship between alanine & tryptophan?
Definition
alanine yields pyruvate directly on transamination with alpha-ketoglutarate & the side chain of tryptophan is cleaved to yield alanine & thus pyruvate
Term
7 amino acids who have portions of their carbon skeletons that yield acetyl-CoA and/or acetoacetyl-CoA (which is converted to acetyl-CoA)
Definition
1. tryptophan
2. lysine
3. phenylalanine
4. tyrosine
5. leucine
6. isoleucine
7. threonine
Term
What is the relationship between phenylalanine hydroxylase & phenylketonuria (PKU)?
Definition
a genetic defect in phenylalanine hydroxylase (the first enzyme in the catabolic pathway for phenylalanine) is responsible for the disease PKU (the most common cause of elevated levels of phenylalanine in the blood)
Term
phenylpyruvate
Definition
yielded when phenylalanine undergoes transamination with pyruvate
Term
What is the relationship between alkaptonuria & homogentisate dioxygenase?
Definition
an inheritable disease of phenylalanine catabolism (alkaptonuria) is caused by a defect in the enzyme homogentisate dioxygenase
Term
2 amino acids which contain five adjacent carbons & a sixth carbon attached through a nitrogen atom
Definition
1. arginine
2. histidine
Term
branched-chain alpha-keto acid dehydrogenase complex
Definition
catalyzes oxidative decarboxylation of all three alpha-keto acids, in each case releasing the carboxyl group as CO2 & producing the acetyl-CoA derivative
Term
maple syrup urine disease
Definition
named because of the characteristic odor imparted to the urine by the alpha-keto acids, it results from a defective branched-chain alpha-keto acid dehydrogenase complex
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