Amino acid metabolism and disorders — Flashcards

Flashcard 11: Give the essential, ketogenic amino acids.

Answer: Leu, Lys

Flashcard 12: Give the acidic amino acids.

Answer: Asp, Glu

Flashcard 13: Give the basic amino acids.

Answer: Arg, Lys, His (but His uncharged at normal pH)

Flashcard 14: What amino acids are used to transport ammonium around the body?

Answer: alanine, glutamate

Flashcard 15: Give the sequence of catelcholamine synthesis.

Answer: Phenylalanine, tyrosine, dopa, dopamine, NE, Epi

Flashcard 16: phenylketonuria (PKU)

Answer:

Extra: mental retardation, growth retardation, seizures, fair skin, eczema, musty body odorlimit phenylalanine in diet and increase tyrosine in dietARphenylalanine hydroxylase

Flashcard 17: maple syrup urine disease

Answer:

Extra: blocked degradation of branched amino acidsin infant: severe CNS defects, mental retardation, death, urine smells like maple syrupARα-ketoacid dehydrogenase

Flashcard 18: homocystinuria

Answer:

Extra: mental retardation, osteoporosis, tall stature, kyphosis, lens subluxation, atherosclerosishomocystinuriavaries depending on specific defect; dietary supplementation of one or more of: cystiene, B6, folate, B12, ARcystathione synthase or homocystiene methyltransferase

Flashcard 19: How many kcal are produced per gram of protein?

Answer: 4 kcal

Flashcard 20: carnitite deficiency

Answer:

Extra: inability to transport long chain fatty acids into mitochondriaweakness, hypotoniahypoketotic hypoglycemia