Biochem Qs wrong/ Genetics Qs wrong Flashcards
In prolonged fasting, what is allosteric activator of liver converting pyruvating forming substances into glucose?
Acetyl CoA
Need to convert PEP to pyruvate by Biotin Dependent Carboxylation to Oxaloacetate in mitochrondria w/ Pyruvate Carboxylase which is increased w/ ACetyl CoA in TCA
Who gets and what causes Brown Pigment stones? (gallstones)
Pigment stones common in rural ASia and increased in women and elderly
Brown pigment secondary to infection of biliary tract and results in release of Beta-Glucuorindase by injured hepatocytes and bacteria –> hydrolysis of bilirubin glucuronides and increase unconjugated bilirubin in bile
ECOLI ASCARIS or LIVER FLUKE Opishorchis sinensis increases risk of brown pigment stone
23 yo female has infant w/ vomiting and lethargy
Hypertonicity and muscle rigidity
Burnt sugar in diapers
What AA needs to be restricted in infant’s diet?
Leucine! Maple Syrup Urine Disease
Defective breakdown of branched AA - Leucine, Isoleucine, and Valine
Defect in Alpha-KetoAcid Dehydrogenase
increased serum branched Alpha ketoacids –> neurotoxicity
Autosomal recessive
5 yo w/ cognitive impairment, hebavioral problems and anemia.
80 yo house
What enzyme susceptible to toxin exposure
ALA Dehydratase and Ferrochelatase enzymes sensitive to lead!!!
Both enzymes contian Zinc
Get accumulations of Aminolevulinic Acid and Protoporphyrin 9 and decreased production of heme –> Microcytic Hypochromic Anemia secondary to lack of Hemoglobin
What does FAB GUT refer to?
Fructose is to Aldolase B
as Galactose is to UridylTransferase
More serious defects lead to PO4 depletion
ATP Production in different tissues - how much and what process?
32 ATP net in Malate Aspartate shuttle in heart and liver
30 ATP in Glycerol-3-POhos shuttle in muscle
Anareoibic glycol makes 2 ATP / glucose
What are the uses of NAD and NADPH
NAD+ Catabolic processes and carry reducing equib like NADH
NADPH - Anabolic processes - steroid and FA synthesis as a supply of reducing agents
Anabolic, Resp Burst, Cytochrome P450, Glutathoine Reductase
Product of HMP Shunt/PPP
Glucokinase vs Hexokinase
Glucokinase - Liver and Beta cells, higher Km (lower affinity), but higher capacity
- Insulin Induced and no feedback inhibition
Gene mutation in MODY!
Hexokinase - tissues, lower Km so higher affinity but lower capacity and Vmax
Not induced by insulin
*Inhibitied by G6P feedback
