Catabolism Purines/Pyrimidines Flashcards
(21 cards)
Enzymes of purine degradation
Adenosine deaminase - ADA
purine nucleoside phosphorylase - PNP
xanthine oxidase
xanthine oxidase location
liver
intestine
xanthine oxidase contents
Molybdenum
2 FAD
2 Fe-S centers
xanthine oxidase inhibition
allopurinol
Purine recycled
salvage pathway, 90%
Purine Salvage Pathway
IMP
GMP
AMP
Hyperuricemia causes
purine degradation decreased uric acid clearance lactic acidosis excess alcohol intake high meat/seafood intake renal insufficiency
Gout
Hyperuricemia
precipitation sodium urate crystals
inflammation great toe
Lesch-Nyhan Syndrome
X-linked
Hyperuricemia
self-mutilation
neurological symptoms
Lesch-Nyhan Syndrome
Allopurinol - XO inhibitor
Gout treatment
Alkalinize urine Colchicine Uricosuric drugs - Probenecid Allopurinol NSAIDS avoid meat and alcohol
Allopurinol
Xanthine oxidase competitive inhibitor
suicide inhibitor
covalent binding
Severe Combined Immune Deficiency
adenosine --X--> inosine adenosine deaminase (ADA) deficiency
SCID
lack cell-mediated and humoral immunity of B and T-cells
inhibit RNA reductase
accumulation dATP
SCID treatment
injections PEG-ADA
PNP-deficiency
less severe SCID
absence T-lymphocytes
SCID disorder
Gene: adenosine deaminase
Metabolite: deoxyadenosine
ADA deficiency
Gout disorder
Gene: multiple
Metabolite: uric acid
hyperuricemia
Immunodeficiency disease
Gene: Purine nucleoside phosphorylase
Metabolite: purine nucleosides
PNP deficiency
Lesch-Nyhan Syndrome
Gene: HGPRT
Metabolite: Purines, uric acid
purine salvage
Hereditary orotic aciduria
Gene: UMP synthase
Metabolite: Orotic acid
pyrimidine synthesis
