Carbohydrates Metabolic Pathways: Generation of Energy Flashcards
breakdown of glucose into 2 pyruvate
molecules to form ATP (Embden Meyerhoff Pathway)
Glycolysis
conversion of non-glucose hexoses (like mannose, fructose and galactose), lipids, amino acids to form more ATPs
Gluconeogenesis
synthesis of glycogen (stored in the liver and muscles) from excess glucose. (INSULIN)
Glycogenesis
Glycogenesis is stimulated by what hormone
INSULIN
breakdown of glycogen to glucose for ATP production
Glycogenolysis
Gluconeogenesis:
____ + a-ketoglutarate —>____ + glutamate
Alanine; pyruvate
Gluconeogenesis:
____ + a-ketoglutarate —> ____ + glutamate
Aspartate; oxaloacetate
Most human tissues cannot utilize ____ and ____
galactose & fructose
The both phosphorylates fructose to fructose-6-
PO4
Fructokinase & hexokinase
has a much higher affinity for glucose than fructose
hexokinase
utilizes fructose-1,6 biphosphate as substrate
Aldolase
It lacks of fructokinase
Fructosuria
Is benign and asymptomatic
Fructosuria
It may be misinterpreted as glucosuria, (positive reducing sugar test)
Fructosuria
It lacks of aldolase (F-1,6
biPO4 cleavage)
Fructose Intolerance
In Fructose Intolerance what happens upon sucrose or fructose intake
Hypoglycemia & vomiting
is obtained from milk sugar.
galactose
The most important organs that can metabolize galactose
liver and erythrocytes
The 2 enzyme defects resulting to galactosemia
- Lack of galactokinase
- Lack of galactose-1-phosphate uridyl transferase
the synthesis of glycogen from monosaccharides
GLYCOGENESIS
Main storage for glycogen
Liver, muscle cells
Glycogen storage in normal tissues
300-320 grams/day
Group of inherited metabolic disorders that involve the lack of or abnormality of enzymes regulating glycogen catabolism and anabolism
Glycogen Storage Diseases
Glycogen Storage Diseases Abnormality in _____ and ____
glycogenesis & glycogenolysis
Accumulation of abnormal glycogen by-products can damage
liver, muscles, heart and other organs
Enzyme Deficiency in VON GIERKE’S
Glucose-6-phosphatase
Enzyme Deficiency in POMPE’S
Alpha-1,4-glucosidase
Enzyme Deficiency in CORI’S
Amylo-1,6-glucosidas
Enzyme Deficiency in ANDERSEN’S
Alpha-1,4-glucan, 6-glucosyl- transferase
Enzyme Deficiency in McARDLE’S
Muscle phosphorylase
Enzyme Deficiency in HER’S
Hepatic phosphorylase
Enzyme Deficiency in TARUI’S
Muscle phosphofructokinase