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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?
Aminotransferases
What 2 AA do not participate in transamination?
Lysine and threonine
What molecule receives the donated amino group (-NH3) in most aminotransferase reaction?
alpha-ketoglutarate
Alanine + Ketoglutarate--> _____
Alpha keto acids and glutamate
What is unique about Aspartate Aminotransferase?
Instead of removing an -NH3 group from an AA it removes a -NH3 group from glutamate to Oxaloacetate yeilding Aspartate and A-Ketoglutarate
What conenzyme is covalently linked to all aminotransferases?
Pyridoxal Phosphate (Derivative of vitamin B6)
What does elevated Aminotransferases mean?
Damage to the tissue which stores them. They are intracellular enzymes
Which enzyme is responsible for releasing the amino group (-NH3) as free ammonia
Glutamate Dehydrogenase
Glutamate ---> Via glutamate dehydrogenase yields ?
Alpha-ketoglutarate, NADH or NADPH, and NH3
What enzyme is responsible for the oxidative deamination?
Glutamate dehydrogenase
What coenzymes does Glutamate dehyrogenase use?
NAD or NADP
Which bacteria are urease Positive?
helicobacter Pylori
Proteus Vulgaris
Proteus mirabilis
Proteus Penneri
Klebsiella
Why are Urease positive organisms of clinical importance?
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.
What is the most common Urea cycle deficiency?
Ornithinie Trans-carbamoylase
What is the inheritance pattern of Ornithinie trans-carbamoylase deficiency?
X-linked
How can NH3 be transported around the body?
Glutamine from most tissues(has 2 -NH3 groups attached to it)

Alanine from muscle
What enzyme is deficient in phenylketonuria? (PKU)
Phenylalanine Hydroxylase (98%)
What does Phenylalanine Hydroxylase do?
converts phenylalanine to tyrosine.
What is Tyrosine used for?
Stimulates melanin production, synthesizes Thyroxine, and NE.
What are the finding of increased phenylalanine in the body?
mental retardation, growth retardation, fair skin, exzema, musty body odor
What is the treatment of PKU?
decrease phenylalanine and increase tyrosine in the diet
What enzyme is defecient in Alkaptonuria
Congenital deficiency of homogentisic acid oxidase
What are this signs of Alkaptonuria?
Black CT, Black urine and alkapton bodies...It's a benign disease
What AA are purely Ketogenic?
Leucine and Lysine