Biochem Pages 82-91 Flashcards
Patient complaints that he can’t smell well and has a persistently rancid metallic taste in his mouth. On physical exam, there is evidence of hypogonadism, sparse pubic, facial, and axillary hair. What deficiency is suspected?
Zinc
2016; Pg 82
Acrodermatitis enteropathica is associated with which vitamin deficiency?
Zinc
2016; Pg 82
Malnourished child presents with a swollen abdomen, anemia, and impaired liver function. What disorder is present?
Kwashiorkor
(2016; Pg 82)
Edema is due to decreased oncotic pressure and liver malfunction to decreased apolipoprotein synthesis
Total caloric malnutrition resulting in tissue and muscle wasting and loss of subcutaneous fat.
Marasmus
2016; Pg 82
Antidote for methanol or ethylene glycol poisoning that Inhibits alcohol dehydrogenase
Fomepizole
2016; Pg 82
Drug given to discourage drinking that inhibits acetaldehyde dehydrogenase
Disulfiram
2016; Pg 83
Effect of chronic alcoholism on NADH/NAD+ ratio in liver
Increase
2016; Pg 83
Chronic intake of _____ is known to induce lactic acidosis, fasting hypoglycemia, and hepatosteatosis.
Ethanol
2016; Pg 83
Cellular site for fatty acid beta oxidation, acetyl-CoA production, TCA cycle, oxidative phosphorylation, and ketogenesis.
Mitochondria
2016; Pg 83
Cellular site for glycolysis, HMP shunt, and the synthesis of steroids, proteins, fatty acids, cholesterol, and nucleotides.
Cytoplasm
2016; Pg 83
Where do heme synthesis, the urea cycle, and gluconeogenesis occur?
Both the mitochondria and cytoplasm
(2016; Pg 83)
HUGs take two
Relocates a functional group within a molecule
Mutase
2016; Pg 84
Co-factor in the transfer of CO2 groups by carboxylases
Biotin
2016; Pg 84
Rate-determining enzyme of glycolysis
Phosphofructokinase-1 (PFK-1)
2016; Pg 84
Fructose-1,6-bisphosphatase is the rate-determining enzyme for which metabolic process?
Gluconeogenesis
2016; Pg 84
5 required cofactors for the pyruvate dehydrogenase complex
TLC for Nancy: 1. Thiamine pyrophosphate (B1) 2. Lipoic Acid 3. CoA (B5, pantothenic acid) 4. FAD (B2, riboflavin) 5. NAD (B3, niacin) (2016; Pg 87)
Increased NAD+/NADH ratio, ADP, and Calcium activates which complex?
Pyruvate dehydrogenase complex
2016; Pg 87
Which is active in the fasting state, FBPase-2 (fructose bisphosphatase-2) or PFK-2 (phosphofructokinase-2)?
FBPase-2
2016; Pg 87
Where do you find glucokinase?
In liver and beta cells of pancreas.
(Hexokinase is in all other tissues)
(2016; Pg 87)
Patient presents with vomiting, rice-water stools, and garlic breath. What co-factor is being inhibited?
Lipoic acid by arsenic
2016; Pg 87
X-linked deficiency in which pyruvate is shunted to lactate (via LDH) and alanine (via ALT)
Pyruvate dehydrogenase complex deficiency
2016; Pg 88
Treatment for pyruvate dehydrogenase complex deficiency
Increased intake of ketogenic nutrients or lysine and leucine
(2016; Pg 88)
Lysine and Leucine are the onLy pureLy ketogenic amine acids
How do NADH electrons from glycolysis enter the mitochondria?
Either via the malate-asparte or glycerol-3-phosphate shuttle
(2016; Pg 89)
Oxidative phosphorylation poison that inhibits ATP synthase
Oligomycin
2016; Pg 89
Antimycin A is an electron transport inhibitor that blocks which complex?
3
2016; Pg 89
CO/CN is an electron transport inhibitor that blocks which complex?
4
2016; Pg 89
Lactating mammary glands, liver, adrenal cortex, and RBCs are sites for what pathway?
HMP shunt (pentose phosphate pathway) (2016; Pg 90)
Heinz bodies (denatured hemoglobin precipitates within RBCs) and bite cells (resulting from phagocytic removal of Heinz bodies) are found in which pathology?
Glucose-6-phosphate dehydrogenase deficiency
2016; Pg 90
Mode of inheritance of disorder in which patient on antituberculosis drugs eating fava beans presents with acute hemolytic anemia
X-linked recessive
(Glucose-6-phosphate dehydrogenase deficiency in which decreased NADPH leads to low glutathione and increased free radicals and peroxides)
(2016; Pg 90)
Pattern of inheritance and symptoms in essential fructosuria
Autosomal recessive, normally a benign condition with fructose appearing in blood and urine
(2016; Pg 91)
Autosomal recessive deficiency of aldolase B resulting in inhibition of glycogenolysis and gluconeogensists
Fructose intolerance (2016; Pg 91)
Patient recently undertaking a juice fast complains of severe vomiting and yellowing of his skin and sclera. Serum studies indicate hypoglycemia. Patient reports other family members are “allergic” to honey. What disorder should be considered?
Fructose intolerance (2016; Pg 91)
One-month old infant is being treated for E.coli sepsis. Physical exam shows jaundice, infantile cataracts, and failure to thrive. What disorder should be considered?
Classic galactosemia
2016; Pg 91
Classic galactosemia is due to absence of _____.
Galactose-1-phosphate uridyltransderase (autosomal recessive disorder)
(2016; Pg 91)
Hereditary deficiency of galactokinase
Galactokinase deficiency (2016; Pg 91)
What contributes to the development of cataracts, retinopathy, and peripheral neuropathy in poorly managed diabetics?
Sorbitol accumulation
2016; Pg 92
The conversion of osmotically active galactitol (via aldose reductase) results from…
High levels of serum galactose
2016; Pg 92
Where in the gut does lactase function?
The brush border
2016; Pg 92
What are the three basic amino acids?
Arginine (Arg), Lysine (Lys), and Histidine (His)
2016; Pg 92
