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20 Cards in this Set

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How are Amino Acids utilized in different nutritional states?
Fed State:
AA carbon --> glucose --> glycogen (energy storage)
AA carbon (excess AAs) --> acetyl CoA (fatty acid) --> triglycerides (adipose tissue) energy storage

Fasting State: AA carbon
-CO2
-pyruvate
-TCA cycle intermediates
-acetyl CoA, acetoacetate
(all for energy production)
Why are amino acids glucogenic, ketogenic or both?
Glucogenic - AA can be converted into glucose
Ketogenic - AA can be converted into ketone bodies
Glucogenic & Ketogenic - AA can be converted into both glucose and ketone bodies
What are the glucogenic AA's?
-Alanine
-Arginine
-Asparagine
-Aspartate
-Cycteine
-Glutamate
-Glutamine
-Glycine
-Proline
-Serine
-histidine
-methionine
-valine
What are the ketogenic AA's?
-leucine
-lysine

(The ones that start with "L")
What are the AA's that are both glucogenic and ketogenic?
-tyrosine
-threonine
-isolucine
-phenylalanine
-tryptophan

(ones that start with "T" plus 2--isolucine & phenylalanine)
What are the essential AA's?
-pretty much all the glucogenic ones except for histidine, methionine, and valine
-tyrosine
What are the main pathways for the synthesis of nonessential AA's?
-AA's synthesized from glycolysis intermediates
-tyrosine
-AA's synthesized from alpha-ketoglutarate
-AA's synthesized from oxaloacetate
What are the characteristic enzyme deficiencies in diseases of AA metabolism?
-Pyridoxal-phosphate ---> transamination & deamination
-tetrahydrofolate ---> metabolism of serine glycine, methionine and histidine
-B12 Vitamin ---> methionine metabolism
-Thiamine-pyrophosphate (B1) and lipoate ---> oxidative decarboxylation of branched chain AA's

(those 4 are derived from vitamins)

tetrahydrobiopterin ---> hydroxylation of phenylalanine, tyrosine, and tryptophan
What are the metabolites that have abnormal levels in diseases of AA metabolism?
look at disease chart on last slide of lecture
What is Homocystinuria?
cystathione synthase, or vitamin B6, B12, or folic acid deficiency
AA metabolism: Methionine
Accum. metaboilites: homocysteine, methionine
Symptoms: myocardial infarction, mental retardation, ectopia lentis
What is the maple syrup urine disease?
a-keto acid dehydrogenase deficiency
AA metabolism: Leu, Ile, Val (I Love Vermont maple syrup)
Accum. metabolites: Leu, Ile, Val and their a-ketoacids
Symptoms: maple syrup odor of urine, neurological problems
treatment: diet restricted in branched chain AA (BCAA)
What is Phenylketonuria?
phenylalanine hydroxylase or tetrahydrobiopterine deficiency
AA metabolism: Phe
Accum. metabolites: Phe, phenyllactate, phenylacetate, phenylpyruvate
Symptoms: musty odor of urine, neurological problems, hypopigmentation
treatment: Phe-restricted, Tyr supplemented diet, avoid aspartame
What is Albinism?
tyrosinase is deficient
AA metabolism: Tyr
Accum. metabolites: none
Symptoms: white skin, hair, pink eyes
What is Tyrosinemia 1?
fumaryl acetoacetate hydrolase deficiency
AA metabolism: Tyr
Accum. metabolites: succinylacetone, fumarylacetoacetate
Symptoms: liver failure, kidney failure
treatment: dietary restriction of phenylalanine and tyrosine
What is Alkaptonuria?
homogentisate oxidase deficiency
AA metabolism: Tyr
Accum. metabolites: homogentisic acid accumulates in urine
Symptoms: urine turns dark, black pigmentation in eye, ear arthritis
What are the major end products of amino acid degradation?
-pyruvate
-TCA cycle intermediates
(oxaloacetate, fumarate, succinyl CoA, a-ketoglutarate)
-acetyl-coa, acetoacetate
Asparaginase is used as what?
antileukemic drug
-asparagine is required for growth of fast dividing leukemic cells

(AA's that are degraded to oxaloacetate)
histidinemia
-disease due to a deficiency in histidase
-elevated levels of histidine in blood and urine
folic acid deficiency
-excrete increased amounts of FlGlu in urine
-FlGlu excretion used to monitor folic acid deficiency
Pellagra
lack of tryptophan and niacin (vit B3) in diet
-4 D's: Dermatitis, Diarrhea, Dementia, and if untreated Death!