Biochemistry: Protein and Nucleotide Metabolism

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Can you name the Biochemistry: Protein and Nucleotide Metabolism?

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

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