Glycolysis Flashcards
Tauri disease
PFK-1 deficiency
How is PFK-1 hormonally regulated
insulin and glucagon
insulin will create protein phosphatases which will remove the P04 from PFK-2 (enzyme) and add it to substrate Fruc-2-6BP. This will activate PFK-1 and favor glycolysis
Glucagon will stimulate kinases which will add a phosphate to PFK-2 making it inactive, not favoring glycolysis
regulation of Pyruvate kinase
high insulin will increase phosphatases which will remove the phosphate from the enzyme adding it to PEP–> pyruvate
high glucagon will increase cAMP which will increase kinases to phophorylate and stop the enzyme from adding the phosphate
Regulation of glycolysis during physical activity
At rest: G-6P inhibits hexokinase; ATP/AMP inhibit PFK-1 and Pyruvate kinase
During exercise: GLYCOLYSIS ACTIVATED ATP/AMP activates PFK, F-1,6,BP will activate pyruvate kinase
Fanconi-Bickle syndrome
• Deficiency of GLUT2 transporters-liver, pancreatic B cells, renal tubules
• Stunted growth
• Hypoglycemia between meals but hyperglycemia during meals
• Hepatomegaly- glycogen buildup in liver due to insulin secretion being low because pancreatic B cells fail to sense an increase in blood glucose levels
treatment is vitamin D and phosphate, uncooked corn startch
Red blood cells and glycolysis
- RBCs lack mitochondria so glycolysis only mechanism for ATP production
- If disrupt glycolysis no energy active transports are messed and cells die anemia
- Men 4-6 million/mm3
- Women 3.5-5.5 million/mm3
v. Hemolytic anemia
• Premature destruction of erythrocytes and could be due to a number of factors
• Membrane (spherocytosis, elliptocytosis)
• Metabolism (nonspherocytic anemia)… leads to less ATP production, less active transporters working, more Na+ in the cell— swelling and hemolysis
• Hemoglobinopathies (thalassemias, sickle cell disease)
• Nutritional deficiencies….vitamin b12/cobalamin
• Viral, bacterial, protozoan infections
CLINICAL MARKERS: elevated lactate dehydrogenase, unconjugated bilirubin
What condition could elevate Phe in fetal brains and disrupt glycolysis by inhibiting PK?
PKU
Potential causes of Diabetes
mutations in GK and mitochondrial tRNAleu genes , aberrant conversion of proinsulin to mature insulin, defective insulin receptor, pancreatitis, trauma, infection, pancreatic carcinoma.