urea cycle (amino acid metabolism)

aspartate

oxaloacetate forms ____ after a transamination reaction

asp, asn, met, thr, iso

oxaloacetate family of amino acids includes:

alanine

pyruvate forms _____ after a transamination reaction

val, ala, leu

pyruvate family of amino acids includes:

ser, cys, gly

3-phosphateglycerate family of amino acids includes:

3-phosphoglycerate dehydrogenase

• diverts 3-phosphoglycerate from glycolysis → 3-phosphohydroxypyruvate

• uses NAD+ as electron acceptor

3-phosphoserine

transamination of 3-phosphohydroxypyruvate gives ____

serine phosphatase

3-phosphoserine + H2O → serine + Pi

ammonia

fish get rid of toxic ammonium through ___

urea

mammals get rid of toxic ammonium through ___

uric acid (to conserve water since urea is water soluble)

birds and reptiles get rid of toxic ammonium through ___

liver (not kidney)

urea cycle occurs in ____ (organ)

mitochondria, cytoplasm

urea cycle occurs partly in ____ and partly in ____ (cellular location)

ammonia, mitochondria

glutamate dehydrogenase reaction converting glutamate → alpha-ketoglutarate releases _____ which transports into ______ from cytoplasm

carbamoyl phosphate

ammonia produced from glutamate dehydrogenase reaction used to make _____ with HCO3- and ATP

carbamoyl-phosphate synthase I

uses ammonia + bicarbonate + 2ATP → carbamoyl-phosphate + 2ADP + 2Pi

ornithine transcarboxylase

• ornithine + carbamoyl-phosphate → citrulline

• old, colorful cats always ask for an umbrella

arginosuccinate synthase

• citrulline + aspartate → arginosuccinate

• old, colorful cats always ask for an umbrella

arginosuccinase

• arginosuccinate → fumarate + arginine

• old, colorful cats always ask for an umbrella

arginase

• arginine → urea + ornithine

• old, colorful cats always ask for an umbrella

fumarate

urea cycle is linked to TCA through ____

10, 90

amino acids from protein provide ___% of energy

carbs and fats provide ___% of energy

protein degradation

important source of energy during starvation or carbohydrate metabolism defects (diabetes)

gluconeogenic

amino acids that are degraded to eventually form glucose

ketogenic

amino acids that are degraded to acetyl-CoA to eventually form fatty acids/ketone bodies

maple syrup urine

• defects in branched chain alpha-keto acid dehydrogenase (BCKDH) causes _____

• valine, leucine, isoleucine are branched chain amino acids

transaminates, SCoA, FAD

• for branched chain amino acids: first ______→ alpha-keto acids then

• branched chain alpha-keto acid dehydrogenase (BCKDH) attaches ____ then acyl-CoA DH uses ____ to oxidize

leucine and lysine

2 purely ketogenic amino acids that are degraded into acetoacetate

phenylalanine and tyrosine

2 amino acids that are degraded to acetoacetate and fumarate

phenyl ketone uria (PKU)

minor product of phenylalanine degradation that accumulates and causes mental retardation unless decrease phenylalanine from diet