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Medical Biochemistry > Amino Acid Metabolism > Flashcards

Amino Acid Metabolism Flashcards

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USMLE® Step 1 flashcards
1
Q

What are the Essential AAs?

A

PVT TIM HALL

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2
Q

How are amino group amino acids removed?

A

Urea Cycle

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3
Q

Keteogenic AAs are broken down to…

A

Acetyl CoA or Acetoacyl CoA

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4
Q

Glycogenin AAs are broken down to…

A
  • Pyruvate
  • 3-PG
  • OAA
  • a-KG
  • Succinyl CoA
  • Fumarate
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5
Q

What is Pyridoxal phosphate?

A

Vitamin B derivative acts as CoEnzyme for transamination reactions

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6
Q

What is the clinical significance of AST/ALT?

A
  • Both are enzymes in transamination rxns
  • Released into blood in Hepatic disease
  • Diagnostic marker
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7
Q

Which organ mainly operates urea cycle?

A

Liver

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8
Q

How does NH3 affect pH?

A

Takes up H+ —> Alkalosis

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9
Q

What happens to glutamine After it reaches liver?

A
  • Converted to glutamate and ammonia

- Ammonia enter urea cycle

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10
Q

What are the possible fates of glutamate?

A
  • Urea
  • Polyamine
  • Glutathione
  • GABA
  • aKG (Glutamate DH)
  • Glutamine (Glutamine Synthetase)
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11
Q

Describe the synthesis of Carbachol Phosphate.

A

NH4+ + HCO3- —> Carbomoylphosphate

  • Uses 2 ATP
  • CSP-I enzyme
  • CSPI is in Mt and is allosteric regulated by N-Acetyl glutamate
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12
Q

What is normal BUN?

A

BUN (Blood Urea Nitrogen) is 10-20 mg/dL normally

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13
Q

What is the Cory Cycle, and it’s significance?

A
  • Glucose-Alanine cycle btwn Muscle and Liver
  • Muscle produces Alanine —> Liver
  • Liver Uses Alanine and a-KG to produce Pyruvate and glutamate
  • Pyruvate made into glucose in liver
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14
Q

What are the main functions of serotonin?

A
  • Regulation of Temp and BP

- Vasoconstrictor and smooth muscle contraction

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15
Q

What is a Phechromocytoma?

A

Tumors of chromaffin tissues:
overproduction of catecholamines,
resulting in hypertension.

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16
Q

What is the pathology of Albinism?

A
  • Tyrosinase deficiency leading to melanin deficiency

- Skin susceptible to sun damage

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17
Q

What are normal Blood creative levels?

A

<1.5 mg/dL

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18
Q

What is the role of Glutathione?

A
  • Maintains protein thiol groups in their reduced state.

* In liver detoxifies xenobiotics (drugs, food additives etc).

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19
Q

What are products and reactants in NO Synthesis?

A

Arginine + O2 + NADPH —> NO + Citrulline

  • NO Synthase
  • THB -> DHB
20
Q

What are clinical correlations associated with AA metabolism in neurotransmission?

A
  • Microglia in Brain immune system
  • MS
  • Epilepsy
  • Plant and snake Poison
  • Organophosphonates
21
Q

What are Ach inhibitors?

A

Physostigmine and Neostigmine

22
Q

What is a clinical correlation for disruption of production of Proline?

A

Scurvy

- Hydroxyproline —> Hydroxylases require Vitamin C

23
Q

Clinical consideration: Phenylalanine metabolism

A
  • Phenylketonuria: Mousy odor, light pigmentation, peculiarities of gait, eczema, dev delay
24
Q

Clinical consideration: Tyrosine degradation

A
  • Accumulation of Homogentisate

- Dark brown polymer in joints —> Arthritis

25
Q

What is produced in methionine metabolism? Significance?

A

SAM - Methylates various compounds in chemical processes like Creatine synthesis, catecholamines synthesis, etc

26
Q

Clinical correlation: Cysteine Synthesis

A
  • Defects in Cystathione synthase result in accumulation of homocysteine and methionine in blood and urine
  • Skeletal deformations, thrombosis, mental retardation
27
Q

Clinical correlation: Sulfur amino acids

A

Cystinuria: Defects in membrane transport of dietary cysteine through epithelial cell
- Forms kidney stones

28
Q

Clinical correlation: Homocysteine

A

Intermediate which Thioesterase LDL—> Atheromas

29
Q

Clinical correlation: Methionine and Branched chain AAs

A

Maple Syrup Urine Disease: Deficiency in Branched chain keto acid DH

  • High in Mennonite Population
30
Q

Clinical Correlation: Methylmalonic acidemia

A
  • Methylmalonyl AoA mutate Missing
  • Conversion of B12 sometimes missing
  • Metabolic Acidosis and mental retardation
31
Q

Clinical correlation: Histidine metabolism

A

Histidinemia:

  • Deficiency Of Histidase
  • Mainly affects skin and Liver
  • Confirmed through skin biopsy
32
Q

Which AA is used to create Carnitine?

A

Lysine

33
Q

Clinical Significance: THF

A
  • Folic Acid (B9)
  • THF is synthesized in certain microorganisms
  • Essential nutrient to humans
  • Inhibited by Sulfonamides
34
Q

Glutamine —> Glutamate

A

Glutaminase

35
Q

What is Vitamin B6, and what reactions is it needed for?

A

Pyridoxal Phosphate:

Decarboxylases:

  • Histamine Production
  • GABA production
  • Dopamine production
  • Cysteine Production: Homocysteine —> Crystathione —> Cysteine (BOTH STEPS)
36
Q

What is Homocystinuria? Differentiate classical and non classical.

A

Build up of Homocysteine

Classical (Enzyme):

  • Crystathione B-Synthase
  • Methionine Synthase (Methionine Resynthesis)

NonClassical:

  • Vitamin B6 (Pyridoxal Phosphate)
  • Vitamin B12 (Methionine Resynthesis)
  • THF Deficiency (Folate Trap)
37
Q

What is Crystathionuria? Differentiate Classical and non classical.

A

Build up of Crystathione

Classical:
- g-Crystathionase

NonClassical:
- Vitamin B6 (Pyridoxal Phosphate)

38
Q

What AA metabolic processes use SAM?

A
  • CAtecholamine synthesis
  • Melatonin Synthesis
  • Creatine Synthesis
  • Carnitine synthesis
  • Cysteine synthesis
39
Q

What is Alcaptonura? What AA is involved?

A

Defect in Homogentisate Oxidase —> Build up of Homogentisate

Tyrosine

40
Q

What is the AA involved in Pellagra? What are important products?

A
  • Tryptophan
  • Produces Niacin —> NMP + ATP —> NAD+
  • Produces Glutarate
41
Q

What is produced in Histidine metabolism?

A
  • N5-Formamino THF

- Glutamate

42
Q

What enzyme is defective in Histidinemia?

A

Histidase

43
Q

What two enzymes are produces from metabolism of 3-PG?

A
  • Serine

- Glycine

44
Q

Which B Vitamin is Folic acid? What product is used to synthesize it? What chemical inhibits production?

A

PABA —> Folic Acid (B9)

Sulfamethoxazole inhibits

45
Q

What is trimethoprim?

A
  • Inhibits DHF —> THF
46
Q

What Are causes of megaloblastic anemia? How is the pathway connected?

A
  • Methionine Synthase deficiency
  • Vitamin B12 Deficiency (Cobalamine)
  • Folate Deficiency (Folate trap!)

N5-N10-MEthylene THF in Folate trap Converst dUMP —> dUTP.

Dysfunction of Conversion can result in DNA trouble!

Medical Biochemistry (26 decks)

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