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24 Cards in this Set
- Front
- Back
Transfer of amino group from one carbon skeleton to another is catalyzed by a family of enzymes called?
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Aminotransferases
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What 2 AA do not participate in transamination?
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Lysine and threonine
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What molecule receives the donated amino group (-NH3) in most aminotransferase reaction?
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alpha-ketoglutarate
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Alanine + Ketoglutarate--> _____
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Alpha keto acids and glutamate
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What is unique about Aspartate Aminotransferase?
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Instead of removing an -NH3 group from an AA it removes a -NH3 group from glutamate to Oxaloacetate yeilding Aspartate and A-Ketoglutarate
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What conenzyme is covalently linked to all aminotransferases?
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Pyridoxal Phosphate (Derivative of vitamin B6)
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What does elevated Aminotransferases mean?
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Damage to the tissue which stores them. They are intracellular enzymes
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Which enzyme is responsible for releasing the amino group (-NH3) as free ammonia
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Glutamate Dehydrogenase
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Glutamate ---> Via glutamate dehydrogenase yields ?
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Alpha-ketoglutarate, NADH or NADPH, and NH3
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What enzyme is responsible for the oxidative deamination?
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Glutamate dehydrogenase
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What coenzymes does Glutamate dehyrogenase use?
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NAD or NADP
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Which bacteria are urease Positive?
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helicobacter Pylori
Proteus Vulgaris Proteus mirabilis Proteus Penneri Klebsiella |
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Why are Urease positive organisms of clinical importance?
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Bc when a patient has decreased kidney function they are unable to remove all the urea from the blood thus more urea is diffused into the intestines where bacteria that are urease positive produce ammonia leading to hyperamonemia.
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What is the most common Urea cycle deficiency?
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Ornithinie Trans-carbamoylase
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What is the inheritance pattern of Ornithinie trans-carbamoylase deficiency?
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X-linked
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How can NH3 be transported around the body?
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Glutamine from most tissues(has 2 -NH3 groups attached to it)
Alanine from muscle |
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What enzyme is deficient in phenylketonuria? (PKU)
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Phenylalanine Hydroxylase (98%)
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What does Phenylalanine Hydroxylase do?
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converts phenylalanine to tyrosine.
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What is Tyrosine used for?
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Stimulates melanin production, synthesizes Thyroxine, and NE.
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What are the finding of increased phenylalanine in the body?
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mental retardation, growth retardation, fair skin, exzema, musty body odor
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What is the treatment of PKU?
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decrease phenylalanine and increase tyrosine in the diet
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What enzyme is defecient in Alkaptonuria
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Congenital deficiency of homogentisic acid oxidase
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What are this signs of Alkaptonuria?
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Black CT, Black urine and alkapton bodies...It's a benign disease
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What AA are purely Ketogenic?
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Leucine and Lysine
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