Biochemistry: Protein and Nucleotide Metabolism

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Can you name the Biochemistry: Protein and Nucleotide Metabolism?

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

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