29 Amino Acid Metabolism 2 Flashcards

1
Q

the non-essential amino acids

A

Alanine, asparagine, aspartate, serine, glycine, glutamine, glutamate, proline, arginine, tyrosine, cysteine (* require an essential AA)

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2
Q

the essential amino acids/ diet

A

Lysine, Isoleucine, leucine, threonine, valine, methionine, Histidine, Tryptophan (Other: Tyrosine and Phenylalanine)

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3
Q

non-essential amino acids made from glycolysis intermediates

A

Serine, Glycine, Cysteine, (Pyruvate)

—->Alanine

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4
Q

non-essential amino acids made from TCA cycle intermediates

A

From Oxaloacetate: Aspartate and Asparagine, From Alpha-ketoglutarate: glutamate, glutamine, proline, Arginine and Ornithine

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5
Q

How Amino acids are synthesized from glycolysis intermediate, 3-phosphoglycerate (4)

A

Serine is synthesized from 3-phosphoglycerate, Glycine is made from Serine in a reversible reaction, Cysteine is made from serine in an irreversible reaction, and Alanine is made from the transamination of pyruvate…..They are all degraded to Pyruvate

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6
Q

synthesis pathway for glycine uses which cofactors

A

It utilizes PLP, a derivative of Vitamin B6, and FH4

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7
Q

most common degradative pathway for glycine

A

glycine is broken down to CO2 using Glycine cleavage enzyme and FH4, and generating NADH and NH4+

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8
Q

the two non-essential amino acids which depend on essential amino acids

A

Tyrosine depends on Phenylalanine, and Cysteine depends on serine for its nitrogen and carbon backbone, and methionine donates its -SH group via homocysteine

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9
Q

Amino acids RELATED to and synthesized from TCA cycle intermediates (alpha-ketoglutarate/glutamate)

A

Glutamate, Glutamine, Proline and Arginine`

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10
Q

Amino acids RELATED to and synthesized from TCA cycle intermediates ( Oxaloacetate)

A

Aspartate and Asparagine

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11
Q

Amino acids degrade and form Oxaloacetate, TCA cycle intermediate

A

Aspartate and Asparagine

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12
Q

Amino acids degrade and form Fumarate,

TCA cycle intermediate

A

Aspartate, Phenylalanine and Tyrosine

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13
Q

Amino acids degrade and form Succinyl CoA,

TCA cycle intermediate

A

Methionine, Isoleucine, Valine and Threonine to succinyl coA via propionyl CoA

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14
Q

Amino acids degraded and form Alpha- ketoglutarate, TCA cycle intermediate

A

Glutamate, Glutamine, Proline, Arginine

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15
Q

the three nitrogen fixing enzymes in humans

A

glutamine synthetase catalyzes conversion of glutamate to glutamine, adding free ammonium to form glutamine
glutamate dehydrogenase catalyzes the reversible trans-amination of glutamate to alpha-ketoglutarate and free ammonium
carbamoyl phosphate synthetase I catalyzes the first step of the urea cycle, the condensation of free ammonium and HCO3- to form carbamoyl phosphate

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16
Q

the enzymes which catalyzes the interconversion of aspartate and asparagine

A

Asparagine synthetase, using ATP and glutamine as the nitrogen donor
and Asparaginase using H20

17
Q

the enzymes which catalyzes the interconversion of glutamate and glutamine

A

Glutamine synthetase, using ATP and NH4+ as the nitrogen donor and Glutaminase using H20

18
Q

Branched chain Amino acids are important because of and the major site of branched chain AA degradation

A

they are important because they are a good source of fuel. they are Valine, Isoleucine and Leucine (VIL). They make up 25% of the aa content of proteins. Muscle is the major site of branched chain-AA degradation

19
Q

defective enzyme of branched chain amino acid degradation in maple syrup urine disease

A

branched chain alpha-keto acid dehydrogenase

20
Q

ketogenic only amino acids

A

Leucine and Lysine

21
Q

both ketogenic and glucogenic amino acids (5)

A

Threonine, Tryptophan, Tyrosine, Phenylalanine, Isoleucine

22
Q

the common form of PKU has which defective enzyme

A

the defective enzyme is phenylalanine hydroxylase enzyme, which is the first step in catalyzing the conversion of phenylalanine to tyrosine

23
Q

the biosynthesis of non-essential amino acids involves which 4 common metabolic intermediates

A

pyruvate
oxaloacetate
alpha-ketoglutarate
3-phosphoglycerate