Biochemistry: Protein and Nucleotide Metabolism

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Can you name the Biochemistry: Protein and Nucleotide Metabolism?

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

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