Benjamin Shared "Biochem Block 2" - 23 Picmonics
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Biochem Block 2
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
- Increased Risk E. Coli Sepsis
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
2 mins
Von Gierke Disease
- Pathophysiology
- Autosomal Recessive
- Type I Glycogen Storage Disease
- Glucose-6-Phosphatase Deficiency
- Signs and Symptoms
- Increased Glycogen in Liver
- Hepatomegaly
- Enlarged Kidneys
- Severe Fasting Hypoglycemia
- Increased Triglycerides
- Increased Uric Acid (Gout)
- Increased Lactic Acid
- Doll-like Facies
- Treatment
- Continuous Oral Glucose (or Cornstarch)
- Avoid Fructose and Galactose
2 mins
Pompe Disease
- Pathophysiology
- Type II Glycogen Storage Disease
- Autosomal Recessive
- Deficiency of Lysosomal Alpha 1,4 Glucosidase
- Acid Maltase
- Signs and Symptoms
- Hypertrophic Cardiomyopathy
- Hepatomegaly
- Macroglossia
- Hypotonia
- Diagnosis
- Increased Glycogen within Lysosomes
- Increased Lactate Dehydrogenase (LDH)
- Increased Creatine Kinase (CK/CPK)
2 mins
Cori Disease
- Pathophysiology
- Type III Glycogen Storage Disease
- Deficiency of Alpha 1, 6 Glucosidase
- Debranching Enzyme
- Limit-Dextrin Accumulation (In Cytosolic Structures)
- Signs and Symptoms
- Hypoglycemia
- Muscle Weakness
- Hypotonia
- Stunted Growth
- Hepatomegaly
- Diagnosis
- Normal Blood Lactic Acid Levels
- Gluconeogenesis Intact
- Increased LFT's
- Increased Creatine Kinase (CK/CPK)
- Treatment
- Continuous Feeding
- High Protein Diet
- Cornstarch (Uncooked)
4 mins
McArdles Disease
- Pathophysiology
- Type V Glycogen Storage Disease
- Muscle Glycogen Phosphorylase Deficiency
- Signs and Symptoms
- Increased Glycogen in Muscle
- Painful Muscle Cramps
- Myoglobinuria
- Normal Lactic Acid
- Treatment
- Vitamin B6
2 mins
Vitamin B1 (Thiamine)
- Mechanism
- Branched-chain Ketoacid Dehydrogenase
- Pyruvate Dehydrogenase
- Alpha-ketoglutarate Dehydrogenase
- Transketolase
- DEFICIENCY EFFECTS
- Wernicke-Korsakoff Syndrome
- Beriberi
- ATP Depletion
- Aerobic Tissues Like Brain and Heart Affected First
- Considerations
- Deficiency Can Worsen with Glucose Infusion
2 mins
Beriberi (Thiamine Deficiency)
- Thiamine Deficiency
- Dry Beriberi
- Polyneuritis
- Muscle Wasting
- Wet Beriberi
- Dilated Cardiomyopathy
- Edema
51 secs
Wernicke-Korsakoff Syndrome
- Thiamine deficiency
- Confusion
- Ophthalmoplegia
- Ataxia
- Korsakoff
- Confabulation
- Personality Changes
- Memory loss
- Alcoholics
- Damage to medial dorsal nucleus
- Damage to mammillary bodies
2 mins
Riboflavin (Vitamin B2)
- Classification
- Vitamin B2
- Biochemistry
- FAD
- FAD Makes 1.5 ATP
- Succinate to Fumarate
- DEFICIENCY
- Glossitis
- Cheilosis
- Corneal Vascularization
2 mins
Vitamin B3 (Niacin)
- Derived from Tryptophan
- Synthesis Requires Vitamin B6
- Constituent of NAD+
- Deficiency Causes
- Hartnup Disease
- Carcinoid Syndrome
- Deficiency Symptoms
- Pellagra
- Diarrhea
- Dermatitis
- Dementia
- Glossitis
- Excess Symptoms
- Flushing
2 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
54 secs
Fructose Intolerance
- Pathophysiology
- Deficiency of Aldolase B
- Fructose 1-P To DHAP And GA
- Accumulation Of Fructose-1-Phosphate
- Inhibition Of Glycogenolysis
- Inhibition Of Gluconeogenesis
- Clinical Features
- Hypoglycemia
- Vomiting
- Jaundice
- Cirrhosis
- Autosomal Recessive
2 mins
G6PD Mechanism
- Turns NADP+ to NADPH
- NADPH Used by Glutathione Reductase
- Detoxifies Free Radicals and Peroxides
- X-linked Recessive
2 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
2 mins
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
4 mins
Fructose Metabolism
- Liver Fructose Metabolism
- Fructose
- Fructokinase
- Fructose-1-Phosphate
- Aldolase B
- DHAP
- Glyceraldehyde
- Glyceraldehyde-3-Phosphate
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
4 mins
Energy Releasing Pathways: Accounting
- Characteristics
- Anaerobic Respiration
- 2 ATP
- 2 NADH
- Aerobic Respiration
- 8 NADH
- 2 FADH2
- 2 ATP
- Oxidative Phosphorylation
- 34 ATP
- 2 ATP Lost
- 36 Net Total ATP
2 mins
Anaerobic Respiration
- Cytosol
- Glucose
- Glycolysis
- Substrate-level Phosphorylation
- 2 NADH
- 2 Net ATP
- 2 Pyruvate
- Fermentation
- NAD+ Regeneration
- Waste Byproduct
2 mins
