Shared Flashcard Set

Details

Biochemistry Test One Part 2
Biochemistry Test One Part 2
197
Biochemistry
Professional
10/30/2012

Additional Biochemistry Flashcards

 


 

Cards

Term
Are Ribosomal Proteins enzymes? Do they Catalyze reactions in Protein Synthesis?
Definition
No NO
Term
Ribosomal proteins associated with small subunit are designated by numbers ____ to ____ (eukaryotes.)
Definition
S1 to S33
Term
Ribosomal proteins associated with the large subunit are ____ to ____ (eukaryotes.)
Definition
L1 to L49
Term
Ribosomal proteins are coded by _____ and therefore _______ is needed.
Definition
mRNAs

RNA polymerase II
Term
In eukaryotes protein synthesis occurs in the _____ and ribosomal proteins are transported to the ____ for binding to the newly synthesized _____ RNA.
Definition
cytoplasm

nucleolus

ribosomal
Term
Intact small and large subunits (___ & ____) are transported out of the ____ to the ____ for translation.
Definition
rRNA and r-protein

nucleus

cytoplasm
Term
In periods when large amount of protein synthesis is required coordination of _____, ______, _____, and messenger RNA for the ribosomal structural proteins must occur.
Definition
RNA polymerase I, II, III,
Term
Prokaryotic protein synthesis begins while ______.
Definition
messenger RNA still being synthesized.
Term
Prokaryotic protein synthesis begins on messenger RNA still being synthesized. This can occur since _______
Definition
there is no barrier such as the nucleus to cytoplasm in eukaryotes.
Term
Binding of the mRNA by_____ in prokaryotes protects the mRNA from ______.
Definition
ribosomes

degradation by nucleases.
Term
Eukaryotic protein synthesis occurs in the cytoplasm. This requires the transport of the ________ from the nucleus.
Definition
large and small ribosomal subunits
Term
________ all associate with the ribosomal ____ subunit to begin the process of protein synthesis.
Definition
Messenger RNA , initiator tRNA and initiation factors

small
Term
major difference between prokaryotic and eukaryotic mRNA is the fact that prokaryotic mRNAs are ______, which means the mRNA ______.
Definition
polycistronic

codes for more than one protein.
Term
____ is the most regulated step in protein synthesis.
Definition
Initiation Step of Protein Synthesis
Term
Protein synthesis is a ____ energy demanding process and control of initiation will control ______.
Definition
high

energy expenditure.
Term
The ____ ribosomal subunit is where initiation begins in both prokaryotes (___S) and eukaryotes (___S).
Definition
small

pro 30S

eu 40S
Term
The small subunit for both prokaryotes (30S) and eukaryotes (40S) is associated with inititiation factors in a pre-initiation complex; ______ in prokaryotes and _____ eukaryotes
Definition
IF1-IF3

eIF-3- eIF-4C
Term
The small subunit will bind the initiator tRNA ____, which is a unique tRNA since it is the only aminoacyl tRNA (one "charged" with an amino acid) that can _____.
Definition
methionine

bind to the P site on the ribosome.
Term
aminoacyl tRNA (______)
Definition
"charged" with an amino acid
Term
Other than the initiator tRNA, all tRNAs bound to the P site contain _____.
Definition
growing peptides. (not just a single amino acid as with initiator tRNA)
Term
The association of the initiator tRNA with the pre-initiation complex requires another initiation factor, ____ for prokaryotes or ____ for eukaryotes. Both of which bind _____
Definition
IF2

eIF2

GTP
Term
Unique to eukaryotes is the requirement for _____ in initiation of protein synthesis, because ____ is required for mRNAs that have a ___ at the 5' end.
Definition
ATP hydrolysis to ADP

Energy

CAP
Term
The 5' mRNA CAP has a compact ____ structure that requires the energy of ATP hydrolysis to be unwound to allow initiation to occur. (Eukaryotes Only)
Definition
secondary
Term
The ____(unwinding) activity of the mRNA 5' CAP is associated with initiation factors ___________.
Definition
helicase

eIF-4A, 4B and 4F.
Term
When the 40S (Small Subunit Eukaryotes) pre-initiating complex has bound: _________ the 40s ____ complex is formed and can bind to the large ribosomal subunit (50S prokaryotes and 60S eukaryotes). This step requires ______
Definition
all necessary initiating factors, initiator tRNA and mRNA

initiation

hydrolysis of GTP to GDP by IF2.
Term
The binding of the intiation complex to the large subunit requires _____ by ____.
Definition
hydrolysis of GTP to GDP by IF2.
Term
Energy comes from hydrolyzing ___ to ____ for charging a tRNA by binding an amino acid.
Definition
ATP to AMP
Term
Energy comes from hydrolyzing ___ to ____ for charging of initiator methionine tRNA. This energy of the attached amino acid and tRNA will be used at ______ bond in the ____ step
Definition
ATP to AMP

peptide

elongation
Term
For both prokaryotes and eukaryotes. associtaion of the small and large subunits requires energy from the hydrolysis of ____ to ____.
Definition
GTP to GDP
Term
Hydrolysis of ___ to ____ for eukaryotes for unwinding the 5' mRNA Cap.
Definition
ATP to ADP
Term
Elongation is a three-step process involving:________ ribosomes, elongation factors (proteins), transfer RNA, messenger RNA and a ribozyme (RNA enzyme)
Definition
ribosomes, elongation factors (proteins), transfer RNA, messenger RNA and a ribozyme (RNA enzyme)
Term
A site for _____.
Definition
aminoacyl tRNA
Term
P site for ____.
Definition
peptidyl tRNA
Term
E site for _____.
Definition
empty tRNA.
Term
At initiation what is the status of the tRNA biding sites of the Ribosome?
Definition
Methionine tRNA is in P site, A and E sites are empty at initiation.
Term
Hydrolysis of ____ to ____ by ___ for association of subunits.
Definition
GTP to GDP by IF2
Term
GTP to GDP by IF2 for association of subunits Is this just for eukaryotes or both prokaryotes and eukaryotes?
Definition
Both
Term
Hydrolysis of ___ to ____ for unwinding CAP on mRNA in eukaryotes catalyzed by _____.
Definition
ATP to ADP

initiation factors eIF-4A, 4B and 4F.
Term
Elongation step 1. P site has initiator met tRNA or a growing peptide chain, E sites is empty at initiation or has a "empty " tRNA (during elongation cycles) and A site will bind incoming aminoacyl tRNA. Energy comes from: ____
Definition
Energy: GTP to GDP by EF-Tu/EF-Ts (prokaryotes) and EF-1α/EF-1β (eukaryotes).
Term
Elongation step 2. Status of A, P, E site.
Definition
P site a growing peptide chain, E sites has a "empty " tRNA and A site has amino acyl tRNA. Peptide bond formation between growing peptide at the P site and the aminoacyl tRNA at the A site.
Term
Peptide bond formation is catalyzed by _____ (__S prokaryotes or ___S eukaryotes ribosomal RNA ), which is called _____.
Definition
ribozyme

23S Pro 28S Eu

Ribozyme named peptidyl transferase.
Term
Elongation step 3. Status of A, P, E site.
Definition
Elongation step 3. A-site is briefly occupied by growing peptide chain with a conformational change in the ribosome and mRNA moves 3 codons in the 5' direction. A-site is then empty, growing peptide chain at the P site and "empty" tRNA is at E site.
Term
Elongation step 3. energy comes from.
Definition
GTP to GDP by EF-G (prokaryotes) and EF-2 (eukaryotes) .
Term
Following the initiation step there are always __ tRNA molecules associated with the ribosomes until the final step of termination when the subunits dissociate.
Definition
2
Term
There is cross talk between the _ site and _ site
Definition
E & A Site
Term
"proof-reading" mechanism of Protein Synthesis is _____
Definition
When the A site is empty and the E site contains a "empty" tRNA molecule the incoming aminoacyl tRNA must bind correctly to the A site resulting in a change in conformation of the ribosomes affecting the E site which results in the ejection of the "empty" tRNA. If an incorrect aminoacyl tRNA comes into the A site, the conformational change does not occur and the aminoacyl tRNA is ejected.
Term
When the A site is empty and the E site _______
Definition
contains a "empty" tRNA molecule
Term
When the A site is empty and the E site contains a "empty" tRNA molecule the incoming aminoacyl tRNA must bind correctly to the A site resulting in a change in conformation of the ribosomes affecting the E site which results in the ejection of the "empty" tRNA.
Definition
Yup
Term
If an incorrect aminoacyl tRNA comes into the A site, what happens?
Definition
The conformational change that ejects the empty tRNA from the T site does not occur and the aminoacyl tRNA is ejected
Term
Proteins synthesized on ribosomes in the cytoplasm are targeted to _____ based on the amino acid sequence within the protein.
Definition
different organelles
Term
Nuclear proteins contain a sequence of amino acid designated as the ______ sequence, that is one or more short sequences of _______, located at the _____ of the sequence.
Definition
nuclear localization

lysines and arginines (positively charged amino acids)

middle
Term
Mitochondria proteins are directed by a ______ sequence, which is composed of a _______.
Definition
mitochondrial localization

alternating pattern of a few hydrophobic amino acids (such as valine, isoleucine, leucine, methionine) and a few positively charged amino acids (lysine and arginine ).
Term
The mitochondrial localization sequences is located ______ and is recognized by _____.
Definition
At the N Terminus

cytoplasmic molecular chaperone.
Term
lysine and arginine are ____ charged.
Definition
positively
Term
Endoplasmic Reticulum (ER) proteins contain a signal sequence at the _____ of the protein consisting of _______.
Definition
9 to 12 hydrophobic amino acids.
Term
Endoplasmic Reticulum (ER) proteins contain a signal sequence at the N terminus of the protein consisting of 9 to 12 hydrophobic amino acids, which is recognized by _________
Definition
signal recognition particle (SRP).
Term
The proteins destined to be in the ER are transported as ______.
Definition
nascent proteins still attached to ribosomes in the act of being synthesized
Term
______ is continuous with the outer nuclear membrane.
Definition
The ER membrane
Term
Proteins directed to the ER either ____ or _____
Definition
reside there or are transported to other organelles such as Golgi, Endosomes and Lysosomes.
Term
ER modifications include: ______
Definition
disulfide bonds formation, addition of carbohydrates (glycosylation) and proteolytic cleavages.
Term
Many ER proteins are modified in the ____ and further modified in the ____.
Definition
ER

Golgi
Term
Golgi Apparatus is Usually located near the _____.
Definition
nucleus
Term
The entry side of the Golgi Apparatus for proteins is referred to as the ____ face and the exit side is the ____ face.
Definition
cis

trans
Term
Gouty arthritis - caused by ______.
Definition
accumulation of a breakdown product of purine nucleotide catabolism - uric acid
Term
Location of 50% of the cases of gouty arthritis _____
Definition
Metatarsal-phalangeal joint at base of big toe ~50% of cases
Term
Several common ______ drugs target specific steps or enzymes associated with nucleotide synthesis
Definition
chemotherapeutic
Term
The sugar is linked to the base by a ______ at the _____ position of the sugar.
Definition
Beta-Glycosidic Linkage

1'
Term
Ribose and Deoxyribose differ by ____
Definition
A Hydroxyl group at the 2' position
Term
Ribose and its derivative deoxyribose are products of the _______.
Definition
pentose phosphate pathway
Term
What has a higher cellular concentration, Ribonucleotides or Deoxyribonucleotides?
Definition
Ribonucleotides
Term
______ cells are primary source of nucleotides in adults
Definition
Liver
Term
nucleotide synthesis levels are extremely low in _____ cells
Definition
neural
Term
Salvage pathway involves the joining of
Definition
Activated Ribose (PRPP) and a base
Term
The _____ salvage pathways are especially important for energy saving and effects of their absence.
Definition
purine
Term
Some tissues (most notably ____ tissue) rely almost exclusively of salvage pathways.
Definition
neural
Term
_____ nucleotides assembled on ribose
Definition
Purine
Term
____ bases pre-assembled, then attached to ribose
Definition
Pyrimidine
Term
Purines are assembled stepwise on C-_’ of _______ which is first activated to form _______.
Definition
1

ribose-5-phosphate

phosphoribosyl pyrophosphate (PRPP)
Term
Transfer of pyrophosphate from ATP to ribose-5-phosphate is catalyzed by ______.
Definition
PRPP synthetase
Term
What is the rate limiting step of nucleotide biosynthesis?
Definition
PRPP synthetase transferring pyrophosphate group from ATP to ribose-5-phosphate
Term
rate limiting steps of biochemical pathways are important steps for ______.
Definition
regulation (eg feedback inhibition)
Term
PRPP can be directed towards: ______.
Definition
Pyrimidine Metabolism

Purine Metabolism

Histidine Metabolism
Term
______ contributes two N to the synthesis of Purines.
Definition
Glutamine
Term
____ contributes almost its entire structure including: _____ to purine synthesis.
Definition
Glycine

N C C
Term
Purine rings are assembled from:_______
Definition
glycine, glutamine, aspartate, N10-formyl-tetrahydrofolate and CO2 (or bicarbonate).
Term
______ is the first purine base constructed
Definition
Inosinate (IMP)
Term
Inosinate (IMP) is the first purine base constructed and _______ bases are derived from inosinate.
Definition
adenine and guanine
Term
The purine base in IMP (Inosinate) is named ______.
Definition
hypoxanthine
Term
What enzyme catalyzes the step that commits to purine synthesis?
Definition
glutamine phosphoribosyl amidotransferase (aka PRPP amidotransferase)
Term
Commitment to purine biosynthesis is catalyzed by glutamine phosphoribosyl amidotransferase (aka PRPP amidotransferase) which takes ______ as a reactant and by adding a ____ from _____, it forms ______.
Definition
PRPP

adds an amine from glutamine by hydrolyzing off pp to the C1' of the ribose and forms phosphoribosylamine
Term
IMP is a branch point between ____ and _____.
Definition
AMP and GMP
Term
IMP to AMP steps?
Definition
Add GTP and Aspartate to IMP to form adenylosuccinate which turns into AMP
Term
IMP to GMP steps?
Definition
Add NAD+ to form XMP (Xanthosine Monophosphate)

Then add ATP and Glutamine to XMP to form GMP
Term
_____ is the source of phosphates for formation of di- and triphosphate forms of nucleotides.
Definition
ATP
Term
Formation of which phophate mono di or tri requires a specific enzyme?
Definition
mono to DI
Term
Specific enzymes are required for the formation of each diphosphate, termed _______.
Definition
nucleotide monophosphate kinases (ex: adenylate kinase, guanylate kinase)
Term
A single enzyme with broad specificity, _______, catalyzes the second reaction of Diphosphate to Triphosphate
Definition
nucleotide diphosphate kinase
Term
glutamine phosphoribosyl amidotransferase (aka PRPP amidotransferase) is inhibitted by ______
Definition
IMP, GMP, AMP.
Term
PRPP synthetase is inhibited by ______.
Definition
ADP and GDP
Term
Conversion of IMP to GMP is inhibited by _____.
Definition
GMP
Term
Conversion of IMP to AMP is inhibited by _____.
Definition
AMP
Term
The reciprocal substrate relation whereby ATP is used in the synthesis of GMP and GTP is used in the synthesis of AMP Does what?
Definition
balances the production of these nucleotides
Term
Salvage pathways allows you to regenerate nucleotides from free bases by attaching them to _____.
Definition
PRPP
Term
_______ catalyzes the recovery of hypoxanthine or guanine.
Definition
Hypoxanthine-guanine phosphoribosyl transferase (HGPRT)
Term
HGPRT catalyzes the recovery of ____ or _____.
Definition
hypoxanthine into IMP or guanine into GMP.
Term
How is most adenine is recovered?
Definition
via inosine (IMP), which is recovered from hypoxanthine by HGPRT
Term
_______ Catalyzes the recovery adenine into AMP.
Definition
Adenine phosphoribosyltransferase
Term
_______ catalyzes the recovery of uracil
Definition
Pyrimidine phosphoribosyl transferase

DOES NOT RECOVER cytosine
Term
_____ is the end product of purine degradation in humans.
Definition
Uric acid
Term
Uric acid is the end product of purine degradation in humans, must be excreted in _____.
Definition
the urine.
Term
Uric acid solubility is dependent on several factors including ____ & _____.
Definition
pH and temperature.
Term
Normal uric acid levels in humans are close to _______.
Definition
its solubility level (~7mg/dl in plasma and interstitial fluids).
Term
Normal Range of Uric Acid:

Males: _____ mg/dl

Females: _____ mg/dl
Definition
Males: 2-7.5mg/dl

Females: 2-7 mg/dl
Term
Uric acid levels increase with ______ & ______.
Definition
age and with weight
Term
Elevations in uric acid levels above its solubility level (hyperuricemia) can lead to _______ or _______.
Definition
deposition of uric acid crystals in the urine (kidney stones) or of sodium urate in plasma or interstitial fluids (gout).
Term
following hyperuricemia and deposition of uric acid crystals in the urine results in ______
Definition
kidney stones
Term
following hyperuricemia and deposition of sodium urate in the interstitial fluids results in ______.
Definition
gout
Term
Gouty arthritis is an inflammatory disease caused by _____ deposition in joints, particularly in the _____ where temperatures are lower
Definition
sodium urate

periphery
Term
Diagnosis of Hyperuricemia is arrived at by detection of _______ crystals under polarized light microscopy in aspirate from affected joint.
Definition
negatively birefringent
Term
Diagnosis of Hyperuricemia is arrived at by detection of ______ under skin, often on affected joint
Definition
tophus (deposit of monosodium urate crystals)
Term
Causes of hyperuricemia is basically anything that causes: ________
Definition
increases in purine biosynthesis, degradation or decreases uric acid excretion
Term
Metabolic diseases that result in an increased supply of ribose-5-phosphate would lead to ______
Definition
hyperuricemia
Term
Increased cell _____ would lead to hyperuricemia.
Definition
cell turnover – presumably because purines from these cells must be degraded (eg cancer patients undergoing chemotherapy)
Term
______ renal excretion would lead to hyperuricemia.
Definition
decreased
Term
Genetic causes of hyperuricemia include _____ in PRPP synthase activity
Definition
increased PRPP synthase activity (normally rate-limiting)
Term
PRPP synthase activity is typically rate-_____
Definition
rate-limiting
Term
Genetic causes of hyperuricemia include _____ in HGPRT activity (salvage enzyme)
Definition
Decreased
Term
Partial deficiency of HGPRT activity leads to______
Definition
hyperuricemia
Term
Complete deficiency of HGPRT leads to _____
Definition
Lesch-Nyhan syndrome
Term
Lesch-Nyhan syndrome is characterized by _______.
Definition
spasticity, mental retardation, choreoathetosis and self-mutilation.
Term
Although individuals with Lesch-Nyhan syndrome are hyperuricemic, the neurological symptoms are not caused by uric acid but due to the _______.
Definition
reliance of the brain on the salvage pathway for purine synthesis.
Term
A genetic glucose metabolism disorder, ________, can result in hyperuricemia by leading to ______
Definition
von Gierke Disease

increased activity of the pentose phosphate pathway (More PRPP)
Term
examples of NSAIDs?
Definition
indomethacin & ibuprofen
Term
2 classes of Anti-inflammatory drugs for treating hyperuricemia and gout?
Definition
Colchicine

NSAIDs (eg indomethacin, ibuprofen)
Term
3 Options for the Treatments for hyperuricemia and gout?
Definition
Anti-inflammatory drugs

Uricosuric agents

Inhibition of xanthine oxidase
Term
Uricosuric agents cause _____
Definition
increase renal excretion of uric acid
Term
Inhibition of xanthine oxidase is accomplished by what drug?
Definition
Allopurinol
Term
Inhibition of xanthine oxidase is accomplished by Allopurinol and leads to _______.
Definition
decreased levels of uric acid and increased levels of hypoxanthine and xanthine, more soluble
Term
Allopurinol-Mechanism of Action?
Definition
Allopurinol replaces the usual substrate (Hypoxanthine or Xanthine) of Xanthine Oxidase and binds tighthly non covalently to Xanthine Oxidase, thereby inhibiting it from making Uric Acid.
Term
The anti-inflammatory drug for treating hyperuricemia and gout ______ can also cause gastrointestinal side effects
Definition
Colchicine
Term
How do you treat acute Gouty Arthritis?
Definition
NSAIDs unless they have an allergy, then Colchicine.
Term
How do you treat Chronic Gouty Arthritis?
Definition
Uricosic agent or Allopurinol
Term
ndividuals with chronic gout are generally counseled to alter their diet to avoid ________ foods.
Definition
purine rich foods
Term
excess purines cannot be ____ and must be ______.
Definition
stored

degraded
Term
The pyrimidine ring is assembled from: ________
Definition
glutamine (as a source of ammonia), aspartate and CO2 (or bicarbonate).
Term
Glutamine is converted to _____ before it is added to pyrimidine ring synthesis.
Definition
Carbamoyl phosphate
Term
Carbamoyl phosphate derived from CO2 and Glutamine forms ______ of the pyrimidine ring, while aspartate forms _______
Definition
C2 N3

C4, C5, C6, N1
Term
____ is the first pyrimidine nucleotide
Definition
UMP
Term
Hereditary orotic aciduria results from a deficiency of _____ & can be treated by _____
Definition
deficiency of pyrimidines

oral administration of pyrimidines
Term
The pyrimidine ring is assembled from: ______
Definition
glutamine (as a source of ammonia), aspartate and CO2 (or bicarbonate).
Term
The initial step of pyrimidine biosynthesis is the formation of ______.
Definition
The formation of carbamoyl phosphate from gluatmine and CO2
Term
The initial pyrimidine ring is combined with PRPP to form _______.
Definition
UTP
Term
The initial pyrimidine ring is combined with PRPP to form UTP, which can then become _______.
Definition
TMP or CTP
Term
TMP can be converted into ______.
Definition
dCTP
Term
Hereditary orotic aciduria caused by mutations that affect conversion of ________
Definition
Orotate into OMP into UMP (The First Pyrimidine Ring, which can be transformed into TMP or CTP)
Term
Hereditary orotic aciduria, condition results from ______ and can be treated with ______.
Definition
Condition due to deficiency of pyrimidines and can be treated with oral administration of pyrimidines.
Term
Hereditary orotic aciduria presents with ______.
Definition
Orotic acid (Orotate) crystals (asymptomatic) in urine of newborns.
Term
UMP + ATP ---> UDP + ADP is catalyzed by what enzyme?
Definition
UMP kinase (specific)
Term
UDP + ATP ----> UTP + ADP
Definition
(nucleotide diphosphate kinase (broad range))
Term
CTP is formed by ______
Definition
amination of UTP
Term
CTP is formed by amination of UTP, ____ serves at the source of the amino group.
Definition
Glutamine
Term
Formation of deoxynucleotides is catalyzed by ______.
Definition
ribonucleotide reductase
Term
Ribonucleotide reductase activity is highest when?
Definition
just prior to S-phase in the cell cycle
Term
Ribonucleotide reductase activity is intricately regulated by _____.
Definition
its products
Term
Ribonucleotide reductase activity is negatively regulated by _____
Definition
dATP as a general sensor
Term
In the process of Ribonucleotide reductase converting the 2' Hydroxyl to a hydrogen, what is oxidized?
Definition
NADPH -> NADP+
Term
Expression of Ribonucleotide Reductase is controlled at the level of the ______.
Definition
cell cycle (increases prior to DNA synthesis)
Term
Ribonucleotide Reductase Expression increases prior to ____.
Definition
DNA synthesis
Term
_____ levels control the overall activity of Ribonucleotide Reductase
Definition
dATP, if dATP levels are high, RR activity is decreased
Term
Levels of ______ control substrate selectivity of Ribonucleotide Reductase.
Definition
dCTP, dGTP and TTP
Term
dUMP is formed from dUDP via _____.
Definition
dUTP (cleaved by dUTPase to form dUMP and PPi)
Term
dUMP can be formed by deamination of _____.
Definition
dCMP
Term
dTMP is formed from ______.
Definition
dUMP
Term
dUMP is converted to dTMP by Thymidylate Synthase and in the process ____ is converted to _____.
Definition
Methylene-THF
to
DHF
Term
Methylene-THF is converted back into DHF by ______, which is the RDS of _____.
Definition
DHF Reductase

Thymidylate Synthase
Term
In degradation of pyrimidines is C converted to U or U to C?
Definition
C is converted to U for degradation by deamination
Term
Uracil and Thymine (pyrimidines) in the process of degradation are reduced by ______.
Definition
Dihydropyrimidine DH
Term
Uracil and Thymine (pyrimidines) in the process of degradation are reduced by Dihydropyrimidine DH, a deficiency of this leads to ______.
Definition
Increase in Pyrimidine Levels
Term
In Adenosine deaminase deficiency (ADD) what enzyme is defective?
Definition
Adenosine deaminase
Term
In Familial Orotic aciduria what enzyme is defective? and leads to _____
Definition
Orotate phosphoribosyl-transferase

Accumulation of orotic acid in blood and urine
Term
In Lesch-Nyhan syndrome what enzyme is defective?
Definition
HGPRT
Term
SCID
Definition
severe combined immunodeficiency
Term
ADA deficiency results in a lack of functional ____ and ___ cells.
Definition
T and B
Term
ADA deficiency is deficiency of _____
Definition
Adenosine Deaminase
Term
ADA (Adenosine Deaminase) deficiency results in immunodeficiency as a result of build up of ________.
Definition
dAMP, dADP and dATP. Inhibition of ribonucleotide reductase by dATP is implicated in the immunodeficiency, thereby blocking DNA synthesis
Term
Why is ADA (Adenosine Deaminase) deficiency results in immunodeficiency as a result of build up of dAMP, dADP and dATP and not AMP, ADP, and ATP?
Definition
The Ribose (AMP ADP ATP) side of degradation can be fed through an alt pathway of AMP to IMP (VIA Adenosine Deaminase) to Hypoxanthine to xanthine to uric acid
Term
flurouracil is a suicide inhibitor of _____ and can be used in cancer treament.
Definition
thymidylate synthase (dUMP to dTMP)
Term
Antifolates are analogs of ______, which block thymidylate synthase by inhibiting _____ activity, leading to a depletion of THF.
Definition
dihydrofolate (DHF)

DHF Reductase (takes DHF back to THF for Thymidylate Syntahse to convert dUMP to dTMP)
Term
_____ rings are assembled on a ribose support while _____ rings are synthesized prior to attachment to ribose
Definition
Purine

pyrimidine
Term
Deoxynucleotides are synthesized by _____ of ribonucleotides
Definition
reduction
Term
Chromosomes are globally demethylated during ______.
Definition
gametogenesis
Term
Chromosomes are remethylated during ____.
Definition
embryogenesis
Term
Hypermethylated regions of DNA are organized into _____.
Definition
heterochromatin
Term
Hypomethylated regions of DNA are organized into _____.
Definition
euchromatin
Term
In eukaryotes, only one base (_____) can be modified by methylation.
Definition
cytosine
Term
In eukaryotes, only one base (cytosine) can be modified by methylation. This modification always occurs in the context of ____ dinucleotide.
Definition
CpG
Term
In prokaryotes, base methylation does what?
Definition
protects DNA from digestion by restriction enzymes and functions as a kind of primitive immune system.
Supporting users have an ad free experience!