Reges Shared "Theme 2" - 59 Picmonics

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Theme 2

Citric Acid Cycle (Krebs Cycle)
Characteristics
Acetyl-CoA + Oxaloacetate
Citric Acid
Isocitric Acid
Produces NADH
Alpha-Ketoglutaric Acid
Produces NADH
Succinyl CoA
Produces GTP
Succinate
Produces FADH2
Fumarate
Uses Water
Malate
Produces NADH
Oxaloacetate
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4 mins
Urea Cycle
Mitochondria
N-Acetylglutamate
Carbamoyl Phosphate Synthetase I
NH3 + CO2
Carbamoyl Phosphate
Ornithine Transcarbamylase
Citrulline
Cytosol
Aspartate
Argininosuccinate
Fumarate
Arginine
H2O to Urea
Ornithine
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3 mins
Fructose Metabolism
Liver Fructose Metabolism
Fructose
Fructokinase
Fructose-1-Phosphate
Aldolase B
DHAP
Glyceraldehyde
Glyceraldehyde-3-Phosphate
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2 mins
Glycolysis
Characteristics
Glucose
Hexokinase or Glucokinase
Glucose-6-phosphate
Fructose-6-phosphate
Phosphofructokinase-1 (PFK-1)
Fructose-1,6-bisphosphate
Aldolase
Glyceraldehyde-3-Phosphate
1,3-bisphosphoglycerate
3-phosphoglycerate
2-phosphoglycerate
Phosphoenolpyruvate (PEP)
Pyruvate Kinase
Pyruvate
Pyruvate Dehydrogenase
Acetyl-CoA
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4 mins
Essential Fructosuria
Autosomal Recessive
Defect in fructokinase
Fructose to fructose 1-P
Benign
Fructose appears in blood and urine
Fructose does not enter cells
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53 secs
Classic Galactosemia
Pathophysiology
Autosomal Recessive
Galactose-1-phosphate uridyltransferase is Absent (GALT)
Impaired Galactose-1-P to UDP-Galactose
Galactitol Accumulation in Lens
Signs and Symptoms
Infantile Cataracts
Failure to Thrive
Hepatomegaly
Jaundice
Intellectual Disability (Mental retardation)
Increased Risk E. Coli Sepsis
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2 mins
Galactokinase Deficiency
Autosomal Recessive
Galactose to galactose 1P
Galactose appears in blood and urine
Benign
Infantile Cataracts
Failure to develop a social smile
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2 mins
Alkaptonuria
Autosomal Recessive
Deficiency of homogentisic acid oxidase
In degradative pathway of tyrosine to fumarate
Homogentisic acid harmful to cartilage
Arthritis
Dark Connective Tissue
Urine turns black on standing
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1 min
Phenylketonuria (PKU)
Pathophysiology
Decreased phenylalanine hydroxylase
Decreased tetrahydrobiopterin cofactor
Autosomal Recessive
Tyrosine Becomes Essential
Phenylalanine found in Nutrasweet
Symptoms
Musty or mousy body odor
Growth retardation
Seizures
Intellectual Disability (Mental Retardation)
Hypopigmentation
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2 mins
Hartnup Disease
Pathophysiology
Autosomal Recessive
Neutral amino acids
Defective Transporter
Renal and Intestinal cells
Signs and Symptoms
Causes tryptophan excretion in urine
Leads to Pellagra
Diarrhea
Dermatitis
Dementia
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2 mins
Maple Syrup Urine Disease
Pathophysiology
Defect in alpha ketoacid dehydrogenase
Blocked degradation of branched chain amino acids
Leucine
Isoleucine
Valine
Signs and Symptoms
Seizures
Intellectual Disability (Mental Retardation)
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1 min
Pyruvate Dehydrogenase Deficiency
Characteristics
Acquired from Thiamine Deficiency
Backup of Alanine and Pyruvate
Symptoms and Complications
Neurologic Defects
Lactic Acidosis
Interventions
Ketogenic Nutrients
Lysine and Leucine (Ketogenic Nutrients)
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56 secs
Ornithine Transcarbamylase Deficiency
Pathophysiology
Most Common Urea Cycle Disorder
X-Linked Recessive
Signs and Symptoms
Hyperammonemia
Decreased BUN
Carbamoyl Phosphate is Converted to Orotic Acid
Pyrimidine Synthesis Pathway
Evident in Babies
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3 mins
Cystinuria
Pathophysiology
Defect of Renal Tubular Amino Acid Transporter
C-O-L-A Acronym
Cystine
Ornithine
Lysine
Arginine
Signs and Symptoms
Excess Cystine in Urine
Hexagonal Crystals
Staghorn Kidney Stones
Diagnosis
Cyanide Nitroprusside Test
Treatment
Acetazolamide to Alkalinize the Urine
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3 mins
G6PD Deficiency
X-linked Recessive
Hemolytic Anemia
Inflammatory Response
Fava Beans
Sulfonamides
Primaquine
Anti TB Drugs
Heinz Bodies
Bite Cells
Prevalent Among African Americans due to Increased Malarial Resistance
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2 mins
G6PD Mechanism
Turns NADP+ to NADPH
NADPH Used by Glutathione Reductase
Detoxifies Free Radicals and Peroxides
X-linked Recessive
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2 mins
Adenosine Deaminase Deficiency
Adenosine to Inosine
Excess ATP
Inhibition of Ribonucleotide Reductase
Prevents DNA Synthesis
Decreases B Cells and T Cells
Major cause of SCID
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1 min
Aerobic Respiration
Mitochondrial Matrix
2 Pyruvate
Pyruvate Decarboxylation
2 NADH
2 Acetyl-CoA
Krebs Cycle
6 NADH
2 FADH2
2 Substrate Level ATPs
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1 min
Anaerobic Respiration
Cytosol
Glucose
Glycolysis
Substrate-level ATP Phosphorylation
2 NADH
2 Net ATP
2 Pyruvate
Fermentation
NAD+ Regeneration
Waste Byproduct
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2 mins
Oxidative Phosphorylation
Inner Mitochondrial Membrane
Electron Transport Chain
Oxygen is Final Electron Acceptor
Proton Pump
Proton Motive Force
ATP Synthase
1 NADH 3 ATP
1 FADH2 2 ATP
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2 mins

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