Catabolism Purines/Pyrimidines Flashcards

1
Q

Enzymes of purine degradation

A

Adenosine deaminase - ADA
purine nucleoside phosphorylase - PNP
xanthine oxidase

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2
Q

xanthine oxidase location

A

liver

intestine

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3
Q

xanthine oxidase contents

A

Molybdenum
2 FAD
2 Fe-S centers

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4
Q

xanthine oxidase inhibition

A

allopurinol

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5
Q

Purine recycled

A

salvage pathway, 90%

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6
Q

Purine Salvage Pathway

A

IMP
GMP
AMP

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7
Q

Hyperuricemia causes

A
purine degradation
decreased uric acid clearance
lactic acidosis
excess alcohol intake
high meat/seafood intake
renal insufficiency
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8
Q

Gout

A

Hyperuricemia
precipitation sodium urate crystals
inflammation great toe

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9
Q

Lesch-Nyhan Syndrome

A

X-linked
Hyperuricemia
self-mutilation
neurological symptoms

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10
Q

Lesch-Nyhan Syndrome

A

Allopurinol - XO inhibitor

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11
Q

Gout treatment

A
Alkalinize urine
Colchicine 
Uricosuric drugs - Probenecid
Allopurinol 
NSAIDS
avoid meat and alcohol
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12
Q

Allopurinol

A

Xanthine oxidase competitive inhibitor
suicide inhibitor
covalent binding

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13
Q

Severe Combined Immune Deficiency

A
adenosine --X--> inosine 
adenosine deaminase (ADA) deficiency
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14
Q

SCID

A

lack cell-mediated and humoral immunity of B and T-cells
inhibit RNA reductase
accumulation dATP

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15
Q

SCID treatment

A

injections PEG-ADA

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16
Q

PNP-deficiency

A

less severe SCID

absence T-lymphocytes

17
Q

SCID disorder

A

Gene: adenosine deaminase
Metabolite: deoxyadenosine
ADA deficiency

18
Q

Gout disorder

A

Gene: multiple
Metabolite: uric acid
hyperuricemia

19
Q

Immunodeficiency disease

A

Gene: Purine nucleoside phosphorylase
Metabolite: purine nucleosides
PNP deficiency

20
Q

Lesch-Nyhan Syndrome

A

Gene: HGPRT
Metabolite: Purines, uric acid
purine salvage

21
Q

Hereditary orotic aciduria

A

Gene: UMP synthase
Metabolite: Orotic acid
pyrimidine synthesis