Biochemistry: Protein and Nucleotide Metabolism

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Can you name the Biochemistry: Protein and Nucleotide Metabolism?

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ClueAnswer
The deficient enzyme in alkaptonuria breaks down tyrosine to form _____________.
Aminotransferase put the amino group from alanine on alpha-ketoglutarate to form glutamate and ___________________.
A deficiency of this enzyme would be indicated by Argininosuccinic Acidemia
This type of base is sythesized piece by piece directly on the ribose
PRPP synthase is negatively regulated by ________________
Lacking OMP decarboxylase or orotate phosphoribosyltransferase causes this rare metabolic condition. Patients often have aneamia, leukopenia and retarded development.
________________ cause poor myelination and mental retardation.
Purine nucleosides are converted to free bases by what enzyme?
This disease causes increased levels of histidine in the blood and urine and causes mental retardation
Condition when uric acid crystals precipitate out of the blood
HGPRT ___________________ causes an increase in the rate of purine synthesis. (Deficiency/Hyperactivity)
Lysosomal proteases that degrade protein in the lysosomal pathway
Most de novo nucleotide synthesis occurs where?
What enzyme converts ribonucleotides to deoxyribonucleotides
This amino acid is used in the production of melanin
What enzyme is deficient in alkaptonuria?
What are uric acid crystals interacting with in gouty arthritis?
This enzyme can convert Adenosine to Inosine
Normal BUN:Creatinine level is ____________:1
What immune cell carries out de novo synthesis of nucleotides?
________________ have a maple/cheesy smell.
Alanine, glycine, methionine, serine, threonine and valine are which type of N-terminal residues?
This enzyme phosphorylates CPS-II to change its allosteric properties making it more easily activated
With what disease would a patient have high levels of valine, leucine and isoleucine in their blood?
Segments rich in what amino acids are rapidly degraded? (acronym)
Pyrophosphates are the basis of this drug class which is used to treat osteoporosis.
People with hyperammonemia are given ________________ so the body uses it for energy of protein
Phenylpyruvate and phenylacetate are _____________.
Ribose-5-Phostphate is an intermediary in what pathway?
What amino acid donates an amino group to PRPP?
A polymer of this chemical creates a black-brown pigment that deposits in connective tissue and can be seen in standing urine in people with alkoptonuria.
Enzyme that transaminates alanine
This enzyme combines ornithine and carbamoyl phosphate to make citrulline
This type of base is assembled completely first then attached to a ribose
The most commonly inherited urea enzyme deficiency
What molecule is at the branch point of de novo purine synthesis?
This enzyme converts 5-phosphoribosyl-1-pyrophosphate to 5-phosphoribosyl-1-amine
Treatment for Lesch-Nyhan Sydrome?
The glycine residue at the carboxyl end of ubiquitin attaches to what residue on the target protein?
Presence of creatine kinase in the blood can indicate ___________________.
This enzyme adds an ammonium ion to glutamate to make glutamine
This enzyme catalyzes the formation of IMP and GMP from Hypoxanthine and Guanine and PRPP
Enzyme that converts thymidine to TMP
If the CK found in the heart is greater than ______ U/L then an MI is probable.
What amino acids are purely ketogenic?
Elimination of an amino group
In hereditary hyperammonemia the administration or citrulline and arginine is usually effective except in the case of _______________.
ClueAnswer
Branched chain ketoacid dehydrogenase requires what cofactor?
A muscle cell that is fully charged with CP has a __________:1 ratio of CP:ATP
What amino acid is added to IMP to make GMP?
Deamination occurs primarily on this amino acid because it is the end produce of many transamination reactions.
This enzyme converts dihydrofolate to tetrahydrofolate
This single bifunctional enzyme has the properties of orotate phosphotranfserase and OMP decaboxylase
The methyl group needed for the conversion of dUMP to dTMP comes from...
A deficiency of this enzyme would be indicated by Argininemia
This enzyme converts UTP to CTP
CPS-II is most sensitive to PRPP during what cell phase?
What enzyme synthesizes PRPP?
This transaminase enzyme helps the urea cycle by being involved in the recycling of fumarate to aspartate.
Enzyme that converts dUMP to dMP
This molecule activtivates CPS-II
A genetic mutation leading to a deficiency in HGPRT leads to what condition?
This enzyme is deficient in histidinemia
This molecule allosterically activates CPS-I
This enzyme combines citrulline and aspartate to argininosuccinate
This genetic disease is characterized by self-mutilating behavior
A genetic mutation in what enzyme can cause increased de novo synthesis of purines and hyperuricemia
This enzyme converts neucleotides to nucleosides
What is the product of the rate limiting step of the urea cycle
This enzyme converts Carbamoyl aspartate to Dihydroorotate
CTP Synthase aminates UTP with an amino group from where?
This isoform of creatine kinase is in skeletal muscle and makes up 95% of the CK in the body.
What type of reaction can create non-essential amino acids from essential amino acids or non-essential AAs from non-essential AAs.
Homocystinuria results from a deficiency of this enzyme
Coverts dihydroorotate to orotate with FAD
Ketogenic amino acids yield which compound(s)?
What amino acid is added to IMP to make AMP?
Bicarbonate, aspartate and ammonia are used to make what?
This molecule inhibits thymidine kinase
This enzyme removes an ammonium ion from glutamate to make alpha-ketoglutarate.
This enzyme found in the gut cleaves urea to get carbon dioxide and ammonia.
What is the base in IMP?
Aspartate, Arginine, Leucine, Lysine and Phenylalanine are which type of N-Terminal amino acids?
Creatine phosphate 'buffers' ___________ during exercise so it doesn't significantly drop.
This is the first enzyme to rise following a myocardial infarction and may only rise transiently and normalize in less thatn 3 days.
This enzyme converts carbomoyl phosphate to carbomoyl aspartate
A very high protein diet or starvation would lead to __________________ (induction/repression) of urea cycle enzymes.
How is maple syrup urine disease treated?
During periods of malnutrition, starvation, cachexia and after traumatic injury the nitrogen balance is _________________.
This enzyme converts creatine to creatine phosphate.
This enzyme converts hypoxanthine to xanthine
The commited step of de novo purine synthesis is conversion of PRPP to ____________________.
What type of amino acids are not principally oxidized in the liver?
Cofactor of all aminotransferase reactions because amino group is first transferred to this molecule.
ClueAnswer
Homocystein and methionine levels are increased in this disease that causes mental retardation and dislocation of the lens of the eye.
This molecule inhibits CPS-II
Deficient enzyme in maple syrup urine disease
Increased BUN usually indicates ________________ disease.
In a healthy person ketovaline is broken down into ______________>
CPS-II is most sensitive to UTP during the end of which cell phase?
This enzyme is deficient in albinism.
Lysosomal degradation activity is ______________ during starvation? (Increased/Decreased)
This enzyme catalyzes formation of AMP from adenine and PRPP
Enzyme that converts deoyuridine to dUMP
Since humans can't store purines we must get rid of them in this form
This enzyme converts Xanthine to Uric Acid
This isoform of CK is found in the heart and is important in diagnosing MIs.
What product builds up in ADA deficient SCID that inhibits deoxynucleotide synthesis
GMP, GDP, GTP, AMP, ADP and ATP all inhibit what enzyme?
This enzyme converts Argininosuccinate to Arginine and Fumarate.
Deficiency of this enzyme would lead to increased levels of orotic acid
20% of SCID cases are due to this enzyme deficiency
What substrate is used to synthesize PRPP?
During periods of growth, pregnancy and convalescence the nitrogen balance is _________________.
Converts orotate to OMP
The most effective treatment for SCID
This isoform of creatine kinase is found in smooth muscle and brain tissue and makes up only 1% of total CK.
This enzyme catalyzes the conversion of bicarbonate and ammonia (from glutamine) to carbomoyl phosphate, releasing glutamate.
The cofactor required for transamination reaction is a derivative of ______________.
A deficiency of this enzyme would be indicated by citrullinemia.
Converts OMP to UMP
Transfer of an amino group from one amino acid to an amino acid carbon skeleton or alpha ketoacid.
What is molecule is 'activated ribose'?
This is a common cause of acquired hyperammonemia.
Ezyme that transaminates aspartate
This drug binds to glutamine and is then excreted from the body in order to reduce the ammonia in the body
Creatine phosphate donates its phosphate group to ADP after _________________.
What immune cell uses primarily the salvage pathway for nucleotide synthesis?
This drug binds to glycine in the liver and is excreted in order to reduce ammonia in the body.
This small, basic protein tags proteins for degradation
This amino acid has two amino groups and is thus the most efficient carrier of ammonia
This enzyme phosphorylates CPS-II to change its allosteric properties making it more easily inhibited
One type of this autosomal recessive disease stems from a deficiency of phenylalanine hydroxylase
Enzyme that converts THF to N5, N10-methylene THF
Deamination reactions create this highly toxic chemical that must be removed from the body.
Creatine kinase is only made in muscle cells that are _________________.
In PKU which amino acid becomes essential?
This metabolite inhibites ribonucleotide reductase leading to the inability of cells to synthesize deoxyribonucleotides and therefore DNA.
Glycine, arginine and methionine are used to synthesize what?
Decreased BUN usually indicates ______________ disease.
A deposit of urate crystals in someone with gout

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