Biochemistry Flashcards

Biochemical Reactions and other such nonsense.

1
Q

Why is glucose considered the body’s primary energy source?

A

Glucose is the primary energy source because:

  • It’s readily available from carbohydrate digestion
  • It can be quickly metabolized for energy
  • All cells can use it
  • It can be stored as glycogen for later use
  • It’s essential for brain function
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2
Q

How does the availability of glucose contribute to its role as a primary energy source?

A

Glucose is readily available from carbohydrate digestion, making it a convenient and accessible energy source for the body.

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3
Q

What advantage does glucose have in terms of metabolism speed?

A

Glucose can be quickly metabolized for energy, allowing cells to rapidly access the energy they need.

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4
Q

How does glucose’s versatility contribute to its importance as an energy source?

A

All cells in the body can use glucose as an energy source, making it a universal fuel for cellular processes.

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5
Q

What storage form does glucose take in the body, and why is this important?

A

Glucose can be stored as glycogen in the liver and muscles for later use, providing an energy reserve when glucose is not immediately available from the diet.

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6
Q

Why is glucose particularly important for brain function?

A

Glucose is essential for brain function because the brain primarily relies on glucose for energy and cannot efficiently use other fuel sources.

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7
Q

Where do the main bioenergetic reactions occur in the cell?

A
  • Cytoplasm: Glycolysis
  • Mitochondrial matrix: Krebs cycle, fatty acid oxidation
  • Inner mitochondrial membrane: Electron transport chain, oxidative phosphorylation
  • Endoplasmic reticulum: Lipid synthesis
  • Peroxisomes: Fatty acid oxidation (very long-chain fatty acids)
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8
Q

What bioenergetic process occurs in the cytoplasm?

A

Glycolysis occurs in the cytoplasm.

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9
Q

Which bioenergetic reactions take place in the mitochondrial matrix?

A

The Krebs cycle and fatty acid oxidation occur in the mitochondrial matrix.

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10
Q

What processes are associated with the inner mitochondrial membrane?

A

The electron transport chain and oxidative phosphorylation are associated with the inner mitochondrial membrane.

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11
Q

Where does lipid synthesis primarily occur in the cell?

A

Lipid synthesis primarily occurs in the endoplasmic reticulum.

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12
Q

What specific type of fatty acid oxidation occurs in peroxisomes?

A

Peroxisomes are responsible for the oxidation of very long-chain fatty acids.

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13
Q

Define aerobic metabolism and provide a bioenergetic example.

A

Aerobic metabolism: Requires oxygen for complete oxidation of substrates
* Example: Complete glucose oxidation through glycolysis, Krebs cycle, and electron transport chain.

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14
Q

Define anaerobic metabolism and provide a bioenergetic example.

A

Anaerobic metabolism: Occurs without oxygen
* Example: Lactic acid fermentation during intense exercise

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15
Q

How does the presence or absence of oxygen differentiate between aerobic and anaerobic metabolism?

A

Aerobic metabolism requires oxygen for complete oxidation of substrates, while anaerobic metabolism occurs without oxygen.

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16
Q

What is an example of a complete aerobic metabolic pathway?

A

A complete aerobic metabolic pathway is the oxidation of glucose through glycolysis, the Krebs cycle, and the electron transport chain.

17
Q

In what physiological situation might anaerobic metabolism, such as lactic acid fermentation, occur?

A

Anaerobic metabolism, like lactic acid fermentation, occurs during intense exercise when oxygen supply is insufficient to meet the energy demands of the muscles.

18
Q

What are the major steps and products of glycolysis?

A

Major steps:

  • Glucose → 2 Pyruvate
  • Products:
  • Net production: 2 ATP, 2 NADH
19
Q

Describe the major steps and products of the Krebs cycle.

A

Major steps:

  • Acetyl-CoA → 2 CO2
  • Products per cycle:
  • 3 NADH, 1 FADH2, 1 GTP
20
Q

What are the key components involved in the Electron Transport Chain (ETC)?

A
  • Key components:
  • NADH and FADH2 (electron carriers)
21
Q

List the key enzymes involved in glycogen metabolism and their functions.

A
  • Glycogen synthase: Adds glucose units to glycogen
  • Glycogen phosphorylase: Breaks down glycogen to glucose-1-phosphate
  • Branching enzyme: Creates branch points in glycogen
  • Debranching enzyme: Removes branch points during glycogen breakdown
22
Q

Describe the steps of glycogenesis.

A
  • Glycogenesis steps:
    Glucose → Glucose-6-phosphate → Glucose-1-phosphate → UDP-glucose → Glycogen
23
Q

Outline the steps of glycogenolysis.

A
  • Glycogenolysis steps:
    Glycogen → Glucose-1-phosphate → Glucose-6-phosphate → Glucose (in liver) or pyruvate (in muscle)
24
Q

Provide five clinical examples of metabolism disorders.

A
  • Diabetes mellitus: Impaired glucose metabolism
  • Phenylketonuria: Phenylalanine metabolism disorder
  • Glycogen storage diseases: Impaired glycogen metabolism
  • Fatty acid oxidation disorders: e.g., Medium-chain acyl-CoA dehydrogenase deficiency
  • Urea cycle disorders: e.g., Ornithine transcarbamylase deficiency
25
What metabolic process is impaired in diabetes mellitus?
Glucose metabolism is impaired in diabetes mellitus.
26
Which amino acid's metabolism is affected in phenylketonuria?
Phenylalanine metabolism is affected in phenylketonuria
27
What is an example of a fatty acid oxidation disorder?
An example of a fatty acid oxidation disorder is Medium-chain acyl-CoA dehydrogenase deficiency.
28
Name a specific urea cycle disorder.
Ornithine transcarbamylase deficiency is an example of a urea cycle disorder. The buildup of ammonia in the blood, caused by OTC deficiency can lead to: 1. Elevated ammonia levels lead to hyperammonemia, which is toxic to the brain. 2. Neurological symptoms include lethargy, irritability, and changes in behavior or mental status. 3. Poor feeding and vomiting are common in infants. 4. Developmental delays are seen in children due to the effect on the central nervous system. 5. In severe cases, it also includes altered mental status and coma, especially when OTC is undiagnosed or untreated.
29
What are the main metabolic fates of pyruvate?
* Lactate: Converted by lactate dehydrogenase (LDH), especially under anaerobic conditions. * Acetyl-CoA: Formed by pyruvate dehydrogenase complex, entering the TCA cycle. * Oxaloacetate: Produced by pyruvate carboxylase, used in gluconeogenesis. * Alanine: Generated through transamination by alanine transaminase (ALT). * Ethanol: In some organisms like yeast, pyruvate is converted to ethanol and CO2. * Acetate: Direct conversion to acetate in some pathways. Key factors influencing pyruvate fate: * Oxygen availability * Cell type and metabolic state * Energy charge of the cell * Regulatory enzymes and cofactors
30
What is the metabolic role of lactate dehydrogenase (LDH)?
1. Catalyzes reversible conversion: * Pyruvate + NADH + H+ ⇌ Lactate + NAD+ 2. Key functions: * Maintains anaerobic glycolysis * Regenerates NAD+ for continued glucose catabolism 3. Importance in anaerobic conditions: * Allows ATP production when oxygen is limited * Crucial for skeletal muscle during intense exercise 4. Role in Cori cycle: * Converts lactate to pyruvate in liver * Enables glucose regeneration 5. Metabolic regulation: * Activity increases with anaerobic respiration * Regulated by substrate availability and allosteric modulation 6. Cellular localization: * Present in cytoplasm of most cells 7. Clinical significance: * Elevated levels indicate tissue damage or disease * Used as a non-specific marker for various conditions
31
What are the yields of ATP, NADH, and FADH2 from the complete metabolism of glucose through glycolysis and the TCA cycle?
Glycolysis: * Net ATP: 2 * NADH: 2 Pyruvate to Acetyl-CoA: * NADH: 2 TCA cycle (per glucose): * ATP: 2 (as GTP) * NADH: 6 * FADH2: 2 Total yield: * Direct ATP: 4 * NADH: 10 * FADH2: 2 Oxidative phosphorylation: * Each NADH: ~2.5 ATP * Each FADH2: ~1.5 ATP Theoretical maximum ATP yield: 30-32 ATP per glucose, depending on the NADH shuttle system ## Footnote The actual ATP yield may be lower due to various factors such as proton leakage and transport costs
32
What is the chemiosmotic hypothesis and its key components?
The chemiosmotic hypothesis, proposed by Peter Mitchell in 1961, explains the mechanism of ATP synthesis in cellular respiration and photosynthesis Key components: 1. Proton gradient: Established across a membrane (mitochondrial or thylakoid) 2. Proton pump: Drives protons against their concentration gradient 3. ATP synthase: Enzyme that catalyzes ATP production 4. Electrochemical gradient: Composed of pH gradient (ΔpH) and electrical potential difference (Δψ) 5. Proton-motive force (PMF): Drives ATP synthesis, calculated as PMF = Δψ - (2.3RT/F)ΔpH Process: * Electron transport chain pumps protons across the membrane * Proton gradient forms * Protons flow back through ATP synthase * Energy released drives ATP production from ADP and Pi Importance: * Explains energy production in respiration and photosynthesis * Links electron transport to ATP synthesis * Provides mechanism for energy storage in biomembranes
33
What are the main enzyme complexes of the electron transport chain (ETC) and their functions?
* Complex I (NADH dehydrogenase): 1. Accepts electrons from NADH 2. Transfers electrons to Coenzyme Q 3. Pumps protons to intermembrane space * Complex II (Succinate dehydrogenase): 1. Accepts electrons from succinate 2. Transfers electrons to Coenzyme Q 3. Does not pump protons * Complex III (Cytochrome bc complex): 1. Receives electrons from Coenzyme Q 2. Transfers electrons to Cytochrome c 3. Pumps protons to intermembrane space * Complex IV (Cytochrome c oxidase): 1. Accepts electrons from Cytochrome c 2. Transfers electrons to oxygen (final acceptor) 3. Pumps protons to intermembrane space Additional components: * Coenzyme Q (Ubiquinone): Mobile electron carrier between complexes * Cytochrome c: Mobile electron carrier between Complex III and IV ## Footnote Key points: * Complexes I, III, and IV contribute to the proton gradient * The proton gradient drives ATP synthesis via ATP synthase * Complex II provides an alternative entry point for electrons from succinate
34
What is fatty acid oxidation and what are its key steps?
Fatty acid oxidation (β-oxidation): A metabolic process that breaks down fatty acids to generate energy. 1. Key steps: Activation: Fatty acid + CoA + ATP → Fatty acyl-CoA + AMP + PPi Transport: Carnitine shuttle moves fatty acyl-CoA into mitochondrial matrix β-oxidation cycle (repeats until fatty acid is fully oxidized): a. Dehydrogenation: Forms double bond, produces FADH2 b. Hydration: Adds water across double bond c. Oxidation: Forms ketone, produces NADH d. Thiolysis: Cleaves 2-carbon acetyl-CoA unit 2. End products: * Acetyl-CoA (enters citric acid cycle) * NADH and FADH2 (enter electron transport chain) 3. Energy yield: * Example: Palmitic acid (16 carbons) yields ~129 ATP Regulation: * Controlled by carnitine palmitoyltransferase I (rate-limiting step) Importance: * Major energy source for heart and skeletal muscle * Crucial during fasting or prolonged exercise
35
What controls where fatty acids are activated to CoA?
Length
36
What are the CPT shuttle antiporters and their roles in fatty acid metabolism?
The CPT shuttle antiporters are key components of the carnitine shuttle system, which facilitates long-chain fatty acid transport into mitochondria for β-oxidation: 1. Carnitine:Acylcarnitine Translocase (CACT/CAC): * Also known as SLC25A20 * Located in the inner mitochondrial membrane * Translocates acylcarnitines into the mitochondrial matrix * Exchanges acylcarnitines for free carnitine in an antiport mechanism 2. Function in the carnitine shuttle: * Accepts acylcarnitines formed by CPT1 on the outer membrane * Transports acylcarnitines across the inner membrane * Returns free carnitine to the intermembrane space 3. Mechanism: * Operates via a ping-pong (alternating access) mechanism * Has a single reorienting binding site * Switches between cytosol-facing and matrix-facing conformations 4. Importance: * Essential for β-oxidation of medium and long-chain fatty acids * Critical in high-energy-demanding tissues (heart, skeletal muscle, kidneys) 4. Regulation: * Activity influenced by counter-substrate concentrations * Can perform uniport of carnitine at lower rates
37
What is ketogenesis and what are its key components?
A metabolic pathway that produces ketone bodies from fatty acids and ketogenic amino acids. 1. Key components: * Location: Primarily in liver mitochondria * Main enzymes: * Carnitine palmitoyltransferase I (CPT-1) * 3-hydroxy-3-methylglutaryl-CoA synthase 2 (HMGCS2) * HMG-CoA lyase 2. Major steps: a. Fatty acid transport into mitochondria via CPT-1 b. β-oxidation of fatty acids to acetyl-CoA c. Formation of acetoacetyl-CoA from two acetyl-CoA molecules d. Conversion to HMG-CoA, then to acetoacetate e. Production of β-hydroxybutyrate and acetone 4. Regulation: * Hormonal: Inhibited by insulin, stimulated by glucagon and other catabolic hormones * Transcriptional: PPARα plays a key role in gene expression 5. Conditions promoting ketogenesis: * Fasting or starvation * Low carbohydrate intake * Type 1 diabetes (insulin deficiency) Significance: * Alternative energy source for brain and other tissues * Important during prolonged fasting or carbohydrate restriction