Chp. 37 Flashcards

1
Q

Although synthesized in the Urea Cycle, it is insufficient and hence essential in children

A

Arg

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2
Q

This Ketogenic Amino Acid resembles isoleucine but like the other branched chain amino acids is essential?

A

Leucine (Leu)

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3
Q

This amino acid with an alcohol
side chain can be synthesized from
3-phosphoglycerate?

A

Serine

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4
Q

This simplest of amino acids has 3 pathways of synthesis?

A

Glycine

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5
Q

This amino acid can be synthesized from serine and methionine

A

Cysteine

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6
Q

This Amino Acid is synthesized from serine and homocysteine

A

Cysteine

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7
Q

This Imino Acid is synthesized step-wise from glutamate via a semi-aldehyde?

A

Proline

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8
Q

The degradation of this amino acid goes through a transamination, reduction and then phosphorylation to yield 2-phosphoglycerate?

A

Serine

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9
Q

In the degradation of Glycine, this enzyme yields energy and N5,N10-CH2-F4

A

Glycine cleavage enzyme

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10
Q

As a byproduct of methionine, this is produced and is implicated in cardiovascular disease?

A

Homocysteine

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11
Q

The degradation of this amino acid goes through FIGLU and requires FH4?

A

Histidine

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12
Q

This amino acid can be converted to nicotinamide during degradation

A

Trp

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13
Q

The amino acids asparagine and aspartate are related to this TCA intermediate?

A

Oxaloacetate

OAA

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14
Q

This amino acid is hydroxylated to tyrosine before it is degraded to fumarate?

A

Phenylalanine

Phe

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15
Q

This amino acid is used to make “SAM” and is one of the family which yield Succinyl CoA

A

Methionine

Met

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16
Q

A hydroxylation on this amino acid used to make catecholamines requires BH4?

A

Tyrosine

Tyr

17
Q

This family of amino acids make up 25% of proteins and all are degraded through a common enzyme complex

A

Branched chain AA’s

Valine, Leucine, Isoleucine

18
Q

In MSUD (maple syrup urine disease) this enzyme complex is deficient?

A

Branched chain a-keto acid dehydrogenase

19
Q

In classic PKU, this enzyme is deficient?

A

Phenylalanine hydroxylase

20
Q

In Tryosemia II, this enzyme is deficient?

A

Tyrosine aminotransferase

21
Q

With this Genetic enzyme deficiency, Kidney stones develop?

A

Primary oxaluria (Type I)

22
Q

In Homocysteinemia,
This enzyme is deficient?
Causing homocysteine to accumulate

A

Cystathione b-synthase