Questions and Answers
What are essential amino acids?
Which amino acids are considered ketogenic?
What are the acidic amino acids?
Aspartic acid and Glutamic acid
Name the basic amino acids.
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What is the primary goal of the HMP Shunt?
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What reduces glutathione?
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Hartnup disorder leads to decreased ______ reabsorption.
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What are the effects of Hereditary Fructose Deficiency?
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Match the following conditions with their deficiencies:
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Alkaptonuria is caused by a deficiency in Tyrosine Hydroxylase.
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What is the treatment for Galactosemia?
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What enzyme is deficient in Phenylketonuria?
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Study Notes
Essential Amino Acids
- Essential amino acids cannot be synthesized by the body and must be obtained through diet.
Ketogenic Amino Acids
- Lysine and Leucine are the only ketogenic amino acids, contributing to the production of ketone bodies.
Acidic Amino Acids
- Aspartic acid and Glutamic acid are the two amino acids with "acid" in their names.
Basic Amino Acids
- Histidine, Lysine, and Arginine are categorized as basic amino acids due to their side chains.
Glucogenic Amino Acids
- Glucogenic amino acids can be converted into glucose through gluconeogenesis.
Phenylalanine Pathway
- Phenylalanine is crucial for the synthesis of tyrosine and further derivatives.
Tryptophan Pathway
- Tryptophan is a precursor for serotonin and melatonin, essential for mood regulation and sleep.
Hartnup Disorder & Vitamin B3 Deficiency
- Hartnup disorder results in decreased tryptophan reabsorption, leading to niacin deficiency, necessary for NAD+/NADP+ production.
Histidine Metabolism
- Histidine is converted to histamine with the help of vitamin B6 (pyridoxine).
Glycine Metabolism
- Glycine is converted to porphyrin and heme, utilizing vitamin B6 as a cofactor.
Glutamate Metabolism
- Glutamate is converted into GABA (gamma-aminobutyric acid), a neurotransmitter, in a B6-dependent reaction.
Arginine Metabolism
- Arginine can produce nitric oxide (NO), creatine, and urea, with nitric oxide synthesis requiring tetrahydrobiopterin (BH4).
Maple Syrup Urine Disease
- This condition arises from branched-chain α-ketoacid dehydrogenase deficiency, impairing the breakdown of isoleucine, leucine, and valine, resulting in syrup-smelling urine, intellectual disability, and poor nutrient intake.
HMP Shunt Goal
- The primary goal of the HMP shunt is to produce NADPH, crucial for biosynthetic reactions.
Functions of NADPH
- NADPH is essential for glutathione reduction, fatty acid synthesis, and cholesterol synthesis.
HMP Shunt Enzyme and Location
- The enzyme glucose-6-phosphate dehydrogenase (G6PD) facilitates the HMP shunt, primarily occurring in the cytoplasm of the liver, mammary glands, and adrenal cortex.
Mnemonic for HMP Shunt Location
- Remember HMP as Hepatic, Mammary, and Periphery of Adrenal.
Glutathione Reductase Pathway Goal
- The goal is to reduce oxidized glutathione back to its active form.
Reduction of Glutathione
- NADPH and glutathione reductase are necessary for reducing glutathione.
Oxidative Stress Causes
- Factors causing oxidative stress can be remembered with the acronym SPIN-F.
G6PD Deficiency Memory Tool
- Specific mnemonics can be developed to remember details about G6PD deficiency.
Fructose Metabolism
- Fruits, juice, and honey are primary sources of fructose, which enters metabolic pathways.
Fructose Kinase Deficiency
- A rare condition characterized by a lack of fructose kinase which affects fructose metabolism.
Hereditary Fructose Deficiency
- Also known as aldolase B deficiency, it hampers fructose metabolism leading to severe reactions.
Consequence of Fructose Intolerance
- Fructose-1-phosphate accumulation causes ATP depletion, resulting in hypoglycemic episodes.
Mnemonic for Fructose Intolerance
- "Here, you are Fruct Int-irely by that Aldolase B" serves as a reminder of the condition.
Galactokinase Deficiency
- Characterized by reduced enzyme activity leading to galactosemia, galactosuria, and increased galactitol.
Mnemonic for Galactokinase Deficiency
- "GALAC-tokinase Deficiency" relates to key findings: galactosemia, cataracts, and abnormal eye tracking.
Classic Galactosemia
- Caused by a deficiency in galactose-1-phosphate uridyltransferase; often leads to serious health issues.
Mnemonic for Classic Galactosemia Symptoms
- "CLASSIC" refers to common complications such as sepsis and developmental issues.
Treatment for Galactosemia
- A strict diet avoiding lactose and galactose is vital for management.
Lactose Intolerance
- The condition is associated with normal intestinal mucosa despite symptoms of discomfort.
Alkaptonuria
- Caused by a deficiency in homogentisate oxidase, leading to the accumulation of homogentisic acid and dark pigment in urine.
Enzymes in Albinism
- Tyrosine hydroxylase and other enzymes are affected in albinism, leading to a lack of pigmentation.
Catecholamine Synthesis Pathway Mnemonic
- A mnemonic can aid in recalling the steps in catecholamine synthesis.
Phenylketonuria (PKU) Causes
- Caused by deficiencies in phenylalanine hydroxylase or tetrahydrobiopterin (BH4).
PKU Symptoms Mnemonic
- "MOLD-E" reminds of symptoms: musty odor, low height, dermatitis, and epilepsy.
PKU Treatment
- Involves increasing dietary tyrosine and decreasing phenylalanine intake; aspartame should be avoided.
Glycolysis Location
- Glycolysis occurs in the cytoplasm, converting glucose into pyruvate.
Glycolysis Products
- The process yields 2 pyruvate, 2 ATP, and 2 NADH molecules.
Glucokinase vs. Hexokinase
- Hexokinase has a basic role in glucose metabolism while glucokinase acts as a glucose sensor in the liver and pancreatic cells.
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Description
Test your knowledge on essential and various amino acids with these USMLE Biochemistry flashcards. Understand the classifications such as essential, ketogenic, acidic, basic, and glucogenic amino acids, as well as specific metabolic pathways. Perfect for medical students preparing for the exam.
