Amino Acid Metabolism Flashcards by Cody Sellers

What are the Essential AAs?

PVT TIM HALL

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How are amino group amino acids removed?

Urea Cycle

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Keteogenic AAs are broken down to…

Acetyl CoA or Acetoacyl CoA

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Glycogenin AAs are broken down to…

Pyruvate
3-PG
OAA
a-KG
Succinyl CoA
Fumarate

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What is Pyridoxal phosphate?

Vitamin B derivative acts as CoEnzyme for transamination reactions

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What is the clinical significance of AST/ALT?

Both are enzymes in transamination rxns
Released into blood in Hepatic disease
Diagnostic marker

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Which organ mainly operates urea cycle?

Liver

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How does NH3 affect pH?

Takes up H+ —> Alkalosis

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What happens to glutamine After it reaches liver?

Converted to glutamate and ammonia

- Ammonia enter urea cycle

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What are the possible fates of glutamate?

Urea
Polyamine
Glutathione
GABA
aKG (Glutamate DH)
Glutamine (Glutamine Synthetase)

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Describe the synthesis of Carbachol Phosphate.

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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What is normal BUN?

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

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

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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What are the main functions of serotonin?

Regulation of Temp and BP

- Vasoconstrictor and smooth muscle contraction

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What is a Phechromocytoma?

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

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What is the pathology of Albinism?

Tyrosinase deficiency leading to melanin deficiency

- Skin susceptible to sun damage

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What are normal Blood creative levels?

<1.5 mg/dL

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What is the role of Glutathione?

Maintains protein thiol groups in their reduced state.

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

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What are products and reactants in NO Synthesis?

Arginine + O2 + NADPH —> NO + Citrulline

NO Synthase
THB -> DHB

What are clinical correlations associated with AA metabolism in neurotransmission?

Microglia in Brain immune system
MS
Epilepsy
Plant and snake Poison
Organophosphonates

What are Ach inhibitors?

Physostigmine and Neostigmine

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

Scurvy

- Hydroxyproline —> Hydroxylases require Vitamin C

Clinical consideration: Phenylalanine metabolism

Phenylketonuria: Mousy odor, light pigmentation, peculiarities of gait, eczema, dev delay

Clinical consideration: Tyrosine degradation

Accumulation of Homogentisate

- Dark brown polymer in joints —> Arthritis

What is produced in methionine metabolism? Significance?

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

Clinical correlation: Cysteine Synthesis

- Defects in Cystathione synthase result in accumulation of homocysteine and methionine in blood and urine - Skeletal deformations, thrombosis, mental retardation

Clinical correlation: Sulfur amino acids

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

Clinical correlation: Homocysteine

Intermediate which Thioesterase LDL—> Atheromas

Clinical correlation: Methionine and Branched chain AAs

Maple Syrup Urine Disease: Deficiency in Branched chain keto acid DH * High in Mennonite Population

Clinical Correlation: Methylmalonic acidemia

- Methylmalonyl AoA mutate Missing - Conversion of B12 sometimes missing - Metabolic Acidosis and mental retardation

Clinical correlation: Histidine metabolism

Histidinemia: - Deficiency Of Histidase - Mainly affects skin and Liver - Confirmed through skin biopsy

Which AA is used to create Carnitine?

Lysine

Clinical Significance: THF

- Folic Acid (B9) - THF is synthesized in certain microorganisms - Essential nutrient to humans - Inhibited by Sulfonamides

Glutamine —> Glutamate

Glutaminase

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

Pyridoxal Phosphate: Decarboxylases: - Histamine Production - GABA production - Dopamine production - Cysteine Production: Homocysteine —> Crystathione —> Cysteine (BOTH STEPS)

What is Homocystinuria? Differentiate classical and non classical.

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)

What is Crystathionuria? Differentiate Classical and non classical.

Build up of Crystathione Classical: - g-Crystathionase NonClassical: - Vitamin B6 (Pyridoxal Phosphate)

What AA metabolic processes use SAM?

- CAtecholamine synthesis - Melatonin Synthesis - Creatine Synthesis - Carnitine synthesis - Cysteine synthesis

What is Alcaptonura? What AA is involved?

Defect in Homogentisate Oxidase —> Build up of Homogentisate Tyrosine

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

- Tryptophan - Produces Niacin —> NMP + ATP —> NAD+ - Produces Glutarate

What is produced in Histidine metabolism?

- N5-Formamino THF | - Glutamate

What enzyme is defective in Histidinemia?

Histidase

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

- Serine | - Glycine

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

PABA —> Folic Acid (B9) Sulfamethoxazole inhibits

What is trimethoprim?

- Inhibits DHF —> THF

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

- 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!