Final W4 Flashcards
Dietary amino acids are transported through the __ __ to the liver. Within ___ amino groups from most amino acids are transferred to _____ to form ____. This is then transported into the ___ and releases ___.
circulatory system, hepatocytes, alphaketoglutarate, glutamate, mitochondria, NH4+
____ is a chemical reaction that transfers an amino group to a ___ __ to form a new amino acid. _____ is the amino group acceptor, while the _____ is the amino acid donor. This is catalyzed by an ____. These reactions function to collect amino groups from many different amino acids in the form of ____.
transamination, keto acid, alpha-ketoglutarate, L-amino acid, aminotransferase, L-glutamate
All aminotransferases use ___ ____ as a prosthetic group. PLP is derived from ____ (vitamin B6), and functions as an amino group carrier. ___ ___ is the aminated form and ___ and amino group. PLP is the __ form and ___ the amino group
pyridoxal phosphate, pyridoxine, pyridoxamine phosphate, donates, aldehyde, accepts
Some extrahepatic tissues generate ___, which is toxic and metabolized to ___ using ATP for transport to the liver. Within liver ___, NH4+ and glutamate are released, regenerating glutamate
NH4+, glutamine, mitochondria
catalyzes the release of glutamate and NH4+ through hydrolysis
glutaminase
Vigorously contracting skeletal muscle (____), produces large amounts of _____ through glycolysis and _____ through protein catabolism. The pyruvate can be metabolized to ___ through transamination, then this product is transported to the liver where transamination again produces ___ and ___.
anaerobic, pyruvate, NH4+, alanine, glutamate, pyruvate
in the muscle, ___ ___ catalyzes transfers NH4+ from glutamate to ___ forming alanine and _____. The alanine is transported to the liver via the ___, where __ ___ catalyzes the transfer of NH4+ from alanine to _____ reforming pyruvate and ___. All generate glutamate within liver mitochondria
alanine aminotransferase, pyruvate, alpha-ketoglutarate, blood, alanine aminotransferase, pyruvate, glutamate
Amino groups from many different ____ acids are collected as the amino group of ____, which is transported into the mitochondria. ___ ___ catalyzed by ___ ____ produces free NH4+ and alpha-ketoglutarate
alpha-amino, L-glutamate, oxidative deamination, L-glutamate dehydrogenase
enzymatic transfer of an amino group from an alpha amino acid to an alpha keto acid
transamination
enzymatic removal of an amino group from an alpha amino acid, generating an alpha keto acid and NH4+
oxidative deamination
transamination and oxidative deamination
transdeamination
Aminotransferases are used to detect ___ ___ following infection, excessive alcohol consumption or __ ___. ___ and ____ aminotransferases are in much higher concentration in those with liver damage
liver damage, drug toxicity, alanine, aspartate
a metabolic pathway in the vertebrate liver that synthesizes urea from amino groups and CO2
urea cycle
Urea contains one amino group derived from __ __ and one derived from ___
carbamoyl phosphate, aspartate
___ ___ ___ ___ catalyzes the formation of carbamoyl phosphate from ___, __ and ____.
carbamoyl phosphate synthetase I, NH4+, HCO3, ATP
____ _____ catalyzes the formation of ___ from glutamate and ____
aspartate aminotransferase, aspartate, oxaloacetate
The formation of carbomoyl phosphate requires two ___ steps. First bicarbonate is ___ to form ____ ___. Then nucleophilic attack by ___ displaces the phosphate, generating ____. Phosphorylation of this product forms __ ___
activation, phosphorylated, carbonic-phosphoric anhydride, NH3, carbamate, carbomoyl phosphate
In the first step of the urea cycle, ___ __ catalyzes the formation of ____ from carbomoyl phosphate and ornithine. The carbomoyl group has high ___ potential.
ornithine transcarbamoylase, citrulline, transfer
In step two of the urea cycle, __ ___ catalyzes the formation of ___ from citrulline and _____. This occurs via a ____ reaction between the amino group of aspartate and ___ group of citrulline. It proceeds through a ______ intermediate
arninosuccinate synthetase, arginosuccinate, aspartate, condensation, carboxyl, citrullyl-AMP
In step 3 of the urea cycle, _______ catalyzes cleavage of arginosuccinate forming ___ and ___. The latter is metabolized to ___ and transported to the __ ___ to enter the citric acid cycle
arginosuccinase, arginine, fumarate, malate, mitochondrial matrix
In step 4 of the urea cycle, ___ catalyzes cleavage of arginine releasing __ and regenerating __
arginase, urea, ornithine
The urea cycle requires ___ ATP equivalents. Oxidizing malate to ___ within the citric acid cycle generates ____, which can generate ____ ATP by oxidative phosphorylation each. Interconnections between pathways reduces the __ ___
4, oxaloacetate, NADH, 2.5, energetic cost
___ ___ are transported into the mitochondrial matrix as malate. Malate is then metabolized to __ and returned to the cytosol
reducing equivalents, aspartate
Argininosuccinase deficiency can be treated by providing a surplus of __ in the diet while also restricting total protein ___. This results in ___ of argininosuccinate, which replaces __
arginine, intake, excretion, urea
Ornithine transcarbamoylase deficiency can be treated by protein ___ diet and large amounts of __ and ___. The excess nitrogen accumulates as ____ and _____. Benzoate is metabolized to __ which is excreted, and phenylacetate is metabolized to _____ which is also excreted
restricted, benzoate, phenylacetate, glycine, glutamine, hippurate, phenylacetylglutamine,
