Chapter 12 ( Glycolysis And Pyruvate Dehydrogenases ) Flashcards
Glucose sensor for insulin release in Beta-cells of pancreas ?
GLUT2 along with Glucokinase
Stimulators of GLUT4 translocation in skeletal muscles ?
Insulin
Exercise by 5’AMP-activated kinase
Why does adipose tissue require glucose ?
To form DHAB which is converted to Glycerol phosphate used to store incoming fatty acids as Triglyceride
Phases of insulin secretion by pancreatic Beta-cells ?
Phase 1 : within 15 mins , glucose stimulates the release of preformed insulin
Phase 2 : within several hours , glucose stimulates insulin synthesis at the gene level
Activators and inhibitors of PFK-1 ?
Activators : AMP Fructose 2,6 bisphosphate Inhibitors : ATP Citrate
Arsenate mechanism ?
Inhibits the conversion o Glyceraldehyde 3-phosphate to 1,3 bisphosphoglycerate by mimicking phosphate
The arsenate containing product is water-labile enabling glycolysis to proceed but with no ATP production
What does mutations of glucokinase do ?
Alter the Km for glucose
Mutation that decrease Km result in hyperinsulinemia and hypoglycemia
Mutations that increase Km result in some cases of MODY
C-peptide ?
Short polypeptide connecting A-chain to B-chain in the proinsulin , removed after proinsulin is packaged into vesicles in the golgi apparatus
Enzymes that catalyzes irreversible reactions in glycolysis ?
Glucokinase / Hexokinase
PFK-1
Pyruvate kinase
Pyruvate kinase deficiency characteristics ?
Chronic hemolysis
Increase 2,3BPG which lower HbA affinity to oxygen
Absence of Heinz bodies ( which present in G6PD deficiency
Galactosemia caused by deficiency of which enzymes ? And symptoms ? Screening test in newborns ?
Galactokinase and galactose 1-phosphate uridyltransferase
Symptoms :
Catarct in early life
Vomiting and diarrhea following lactose ingestion
Mental retardation and lethargy
Liver damage and hyperbilirubinemia
Screening test : heal prick test
Mechanism of cataract in galactosemia ?
Transformation of galactose into galactitol by aldose reductase which accumulate in the lens and causes osmotic damage
Why there is no cataract in fructosuria patients ?
Because a fructose is a ketose not an aldose ( doesn’t have aldehyde group for the aldose reductase to work on )
Hereditary fructose intolerance defect and symptoms?
Aldolase B deficiency Symptoms : Lethargy and vomiting Liver damage , hyperbilirubinemia , hypoglycemia Hyperuricemia , lactic acidosis Fanconi syndrome
Coenzymes used by pyruvate dehydrogenase ?
Thiamine pyrophosphate Lipoic acid Coenzyme A FAD(H2) NAD(H)