Nitrogen Metabolism/ Urea Cycle Biochemistry

What is the most prevalent neurotransmitter?

What does glutamate readily converts into?

Glutamate has how many carbons? Aspartate?

Glutaminase degrade _____ to form _____

Glutamine synthetase converts ____ to ____

Ornithine, citrulline and arginine are all compounds formed from ______

What is the source of most of the body’s ammonia?

True/false: half the dry weight of urine is uric acid
True False

What is the committed step of the urea cycle?

Which is the rate limiting step of the urea cycle?

What is carbamoyl phosphate important for?

True/False: energy is used to turn carbonic acid into carbamoyl phosphate
True False

True/False: ammonia released from glutamine or glutamate enter the urea cycle as carbonic acid
True False

How is pyrimidine biosynthesis come about?

What are the four organs involved for nitrogen metabolism?

Where does N2 mostly come from?

Ammonia is converted to which compound when it is getting exported out of the blood?

Liver ____ nitrogen and kidneys _____ them

Which is the inert harmless transporter to move ammonia from brain to the liver?

How many ammonia can each glutamine transport?

What is the function of transaminase?

What enzyme produce glutamine in the brain?

What enzyme produce alanine in muscle?

Which enzymes produce ammonia in liver?

Which enzymes produce ammonia in the kidneys?

In which type of cells do glutamine or glutamate release nitrogen in the form of ammonia?

Where does the urea cycle occurs?

Which cell exports urea into blood for transport to kidneys?

Where does nitrogen metabolism occur/ urea formation?

True/False: kidney is where urea is made
True False

Which three urea cycle enzymes are found in the mitochondrial matrix?

Which three urea cycle enzymes are found in the cytosol?

What results with loss of function mutations of N-acetylglutamate synthase?

True/false: ammonia comes from glutamate/glutamine deamination
True False

Which urea cycle intermediate is formed in the cytosol and transported back to the mitochondrial matrix

How many nitrogens in urea molecule and where did they come from?

True/false: ornithine transcarbamylase deficiency is an X linked dominant disorder
True False

Loss of function is typically _____ mutations

What are some immediate emergent treatment for hyperammonemia?

Why is lactulose the most widely used pharmaceuticals for hyperammonemia?

Rate of ammonia/ammonium drops ____ fold when treating it with lactulose

Which enzyme moves aspartate and glutamate in and out of the mitochondria?

What is the HHH syndrome?