Test3- Diseases and cofactors Flashcards
Carboxlyases use what as a cofactor?
Biotin
What does Methylmalonyl CoA mutase need as a cofactor
Deoxyadenosyl cobalamin (B12)
What is the rate limiting enzyme of lipolysis
Hormone sensitive lipase
Hormone sensitive lipase is ________ when it is on
Phosphorylated
What is the rate limiting enzyme of glycolysis?
PFK-1
What is hexokinase inhibited by
G6P (its product)
What is glucokinase inhibited by?
Nothing, no product inhibition
What is PNDM from?
What pathway is active when PFK-2/FBP-2 is phosphorylated?
Gluconeogenesis
PFK-2 is ________ when active
Dephosphorylated
Pyruvate kinase is active when it is ______
Dephosphorylated
PDHC is active when _______
Dephosphorylated
What happens with a PDHC deficiency?
What happens with a thiamine deficiency? What is the syndrome that can manifest?
What is the rate limiting enzyme of TCA cycle?
Isocitrate dehydrogenase
What happens with arsenic poisoning?
What is LHON?
What is Leigh syndrome? What is the presentation?
What are the inhibitors of the ETC and what are the important examples and treatment?
What are the examples of uncouplers of the ETC and the consequences? What are the natural uncouplers?
What happens with hyperthyroidism?
What happens with atractyloside poisoning?
What is the rate limiting enzyme of glycogenesis?
Glycogen synthase
Where does glycogenesis occur
Cytosol
Where does Liver Glycogenolysis occur
Cytosol
What is the rate limiting enzyme of Liver Glycogenolysis
Glycogen phosphorlyase
What is the cofactor needed by glycogen phosphorylase?
Pyridoxal phosphate B6
Where does gluconeogenesis occur
In the liver and kidney cortex, in mitochondrial matrix and cytosol
What is the rate limiting enzyme of gluconeogenesis?
FBPase-1
What happens with a biotin deficiency?
What happens with a PEPCK deficiency?
What is the rate limiting enzyme of the HMP shunt?
G6PD
Where does the HMP shunt take place?
Cytosolic pathway in all cells
What happens with a GALT deficiency?
What happens with an aldolase B deficiency?
What happens with low insulin levels? What does Niacin inhibit?
What is the rate limiting enzyme for Beta oxidation?
Carnitine shuttle (CPT-I
enzyme*)
Where does Beta oxidation take place
Mitochondria
What is the rate limiting enzyme of ketogenesis?
HMG-CoA synthase
What happens with continuously low I/G ratio?
What is Jamaican vomiting sickness?
What is the rate limiting enzyme of Lipogenesis
Acetyl-CoA carboxylase
Where does Lipogenesis occur
Liver, cytosol on surface of SER
Decrease in bile salts can be from what
Cholestasis from liver or bile duct
Decrease in pancreatic enzymes can be from what
Cystic fibrosis, pancreatitis, EPI
Decreased lipid absorption can be from what
IBD
