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75 Cards in this Set

  • Front
  • Back
difference between a nucleotide and a nucleoside
nucleosides: base and sugar
nucleotide: base, phosphate, and a sugar
linkage that binds a phosphate
ester linkage to Carbon 5 on sugar
two types of sugar
ribose
deoxyribose
difference between the sugars
has an OH on the carbon 2 position instead of a H
linkage that binds the base
N glycosidic bond
name the purines
Adenine and Guanine
name the pyrimidines
Cytosine, Thymine, and Uracil
basic nucleotide funciton
1) DNA Synthesis
2) RNA synthesis
3) transfer of highenergy groups
4) Component enzymes
5) regulatory molecules
purine ring structure
physical notecard
origins of purine ring
1) Aspartate
2) N10 FTHF
3) amide N of glutamine
4) N10 FTHF
5) glycine
6) CO2
Steps in Purine Synthesis
1) R5P
2) PRPP
3) PAR
4) GAR
5) FGAR
6) FGAM
7) AIR
8) CAIR
9) SAICAR
10) AICAR
11) FAICAR
12) IMP
1) Purine
PRPP synthetase
hydrolysis of ATP--> AMP
2) Purine
Glutamine-PRPP amidotransferase
glutamine + H20 --> Glutamate + PP1
3) Purine
adition of glycine
hydrolysis of ATP --> ADP + Pi
4) Purine
addition of N10formyl tetrahydroflourate
goes to THF
5) Purine
Glutamine + ATP --> Glutamate + ADP + Pi
6) Purine
ATP --> ADP _Pi
7) Purine
addition of CO2
8) Purine
addition of aspartate
ATP --> ADP + Pi
9) Purine
fumerate leaves
10) Purine
N10 formyl tetrahedral flourate goes to THF
11) Purine
bye bye H20
IMP pathway
draw pathway
1) IMP
Adenylsuccinate synthetase
IMP--> Adenylsuccinate
addition of aspartate
GTP --> GDP + Pi
regulated by GTP
2) IMP
Adenylsuccinase lyase
bye bye fumerate
adenylsuccinate --> AMP
3) IMP
IMP dehydrogenase
IMP--> XMP
NAD + H20 --> NADH + H2O
4) IMP
GMP synthetase
XMP-->GMP
glutimate + ATP -->glutimine + AMP + PPi
5) IMP
AMP deaminase
H20 --> NH4
adenosine deaminase
H20 --> NH4
6) IMP
GMP reductase
NADPH --> NADP
regulation AMP deaminase
negative : GDP, GTP, Pi
positive: ATP, K+
regulaton of GMP reductase
negative: XMP
positive; GTP
7) IMP
nucleosidase
H2O--> Pi
8) IMP
Purine nucleotide phosphorylase
Pi --> ribose 1- phosphate
produces base
9) IMP
guanine deaminase
H20 --> NH4
10) IMP
Xanthine oxidase
O2 + H2O --> H202
competitive inhibitors
11) HGPRT
12) APRT
11) IMP
Hydroxythanine + PrPP --> IMP + PPi
Guanine + PRPP --> GMP + PPi
IMP and GMP competive inhibitors
12) IMP
Adenine phosphoribosyl transferase
Adenine + PRPP --> AMP + PPi
AMP competitive inhibitor
Deficiencies in purine synthesis
#1 Purine Nucleotide Phosphorylase Deficiency
in enzyme 8
#2 severe combined Immunodeficinecy (SCID) in enzyme 5
#3 Lesch-Nylan Syndrome in enzyme 11
#4 primary gout in Purine pathway enzyme 1
#5 Von Gierkes disease
#6 Xanthurnia
Defiency #1
Purine nucleotide deficiency in enzyme number 8 (lack)
leads in increase in purine nucleotides
decrease uric acid formation
impaired T-cell function
less severe than SCID
Deficiency #2
Severe combined immunodeficiency SCID
enyzme: adenosine deaminase (8) lack of enzyme
leads to both T-Cell and B-cell dysfunction
early-dealth overwhelming infection
gene therary treatment
Deficiency #3
Lesch-Nylan Syndrome
also caused by Gout (deficiency)
enzyme deficiency in HGPRT (11) (lack of HGPRT functional gene)
X-linked

increase purine synthesis
neuroproblems
hyperuricemia
decrease IMP/GMP
increases PRPP

allipurnol
allipurnol
decreases deposition sodium urate crystals
becomes oxidized
to form alloxanthine
which is a negative inhibitor of xanthine oxidase
thus decrease uric formation
Defiency #4
Secondary gout
enzyme Purine 1: PRPP synthetase (increased activity)
causes hyperuricemia
overproduction of purine nucleotides
sodium urate crystals
Hydroxyurea
decreased nucleoside disphosphate reductase
regulation of Glutamine-PrPP amidotransferase
allosterically
inhibited: ATP, ADP, AMP
inhibited: GTP, GDP, GMP
stimulated: PRPP
what happens when ADA is gone
SCID
deoxyadenosine is usually phosphorylated to form dATP
50 fold amounts in lympocytes that contain alot of salvaging exzyme
dATP inhibits ribonucleotide synthetase, preventing dNTP
overall effect is the inhibition of DNA synthesis of the lympocytes
horrible infections
clinical manefestation of Lesch-Nyhan
severe symptoms of gout
severe malfunction of neurosystem because hyperuricemia (NH4+ is neurotoxic)
self-mutilation
death before 20th year
Defiency #5
Von Gierkes
Glucose 6 Phosphotatase
GNG enzyme #4
increase availibility of substrate (glucose 6-phosphate)
increases flux through HMP
increased ribose 5-phosphate
increased PRPP
increased purines
increased uric acid
Deficiency #6
xanthinuria
enzyme: xanthine oxdiase 10
lack
clinical: hyperuricemia, xanthine renal lithiasis
RRPP is used for
1) synthesis of purines
2) synthesis of pyrimidines
3) synthesis of NAD
4) salvage of nucleotide bases
step 1 purines is inhibited by
dinucleotides
ADP
2,3-bisphosphoglycerate
step 1 purines is stimulated by
inorganic phosphate
where did ribose-5-phosphate come from
Hexose monophosphate shunt
three phases of purine synthesis
1) formation of IMP
2) conversion of IMP to GMP
3) conversion of IMP to AMP
regulation of step 2 purines
PRPP amidotransferase
activated by PRPP
inhibited by AMP, GMP, IMP
number of steps between 2 and 11
9 steps
name the purine bases
adenine and guanine
name the pyrimidine bases
cytosine
uracil
thymine
name the purine nucleoside
base-ribose
adenosine
guanosine
name the purine nucleotides
AMP, GMP
what is the nucleotide with the highest concentration
ATP
what is the order of nucleotide, nucleoside, and base prevalane
1) nucleotide
2) nucleoside
3) base
regulation of purine 6
GMP reductase
inhibition: XMP
activation: GTP
regulation of purine 5
GDP, GTP, P inhibition
ATP, K activation
regulation of purine 3
inhibition GMP
regulation of purine 1
inhibition: AMP
GTP stimulates
IMP and AMP
ATP stimulates
IMP, GMP
purine enzyme 9 is also called
guanase
regulation of HGPRTase
GMP, IMP (inhibited)
regulation of ARPTases
inhibited by AMP
sources of nucleotides
1) Amino acids
2) Formate
3) CO2
4) PRPP
purine nucleotide cycle
1) AMP --> IMP
2) IMP--> Adenylosuccinate
3) Adenylosuccinate-->AMP

step 3 gives off a fumerate on the TCA cycle, to regenerate the muscle cells own source of energy
urinary end product of purines
uric acid