Carbohydrates and Lipids Updated Flashcards
Sorbital
sugar alcohol found in patients with diabetes. Those patients can also have cataract. Sorbitol can also be deposited in the schlera of the eye. Sorbital -> carbohydrate -> diabetes-> Cataract.
Energy source (ATP production), storage (glycogen synthesis), glycosylation reactions and precursor for synthesis of other molecules
Glucose (monosaccharides)
Energy source, storage and fatty acid synthesis
Fructose (monosaccharides)
Protein glycosylation reactions
Mannose (monosaccharides)
Energy source after conversion to glucose, milk production during lactation, protein glycosylation in nerves
Galactose (monosaccharides)
Monosaccharides can be joined by ______ to form disaccharides as well as oligosaccharides and polysaccharides
glycosidic bonds
•Note the numbering, e.g., 1-4, indicating the carbon #s
Sucrose
Glucose + fructose (disaccharides of physiological importance)
Lactose
galactose +glucose (disaccharides of physiological importance)
Malotse
glucose + glucose (disaccharides of physiological importance)
polysaccharides of physiological importance
memorize chart, focus on Subunit and bonds
Compound that have the same chemical formula, but different
structures are
isomers
_____ are defined as carbohydrate isomers that differ in the configuration around only one carbon atom
Epimers
d-glucose and d-galactose are epimers at carbon 4. d-glucose and d-mannose are epimers at carbon 2.
Note the differences between glucose and galactose and ribose and deoxyribose
Glucose can form two enantiomeric forms: L-Glucose and D-Glucose. ________ is the predominant form in nature.
D-Glucose
Glucose in solution forms the
pyranose structure
Which polysaccharide is made of b-D-Glucose?
Cellulose
The enzymes that catalyze the reaction are defined as
glycosyltransferases
Note the numbering, e.g., 1-4, indicating the carbon #s
The enzymes that catalyze the reaction are defined as
glycosyltransferases
Note the numbering, e.g., 1-4, indicating the carbon #s
Carbohydrates can be attached by glycosidic bonds to non-carbohydrate molecules such as nucleic acids, lipids and proteins through______; producing glycoproteins and glycolipids respectively
N- or O-glycosidic bonds
Liver glycogen is used for controlling blood sugar levels whereas muscle glycogen is used by the muscles for contraction
Most of the glycogen in the body is stored in the muscle ~ 400g in adults, compared to ~ 100g in liver
A single glycogen molecule can contain up to 55,000 glucosyl residues
Liver glycogen levels are affected by Fed/Fast conditions, while muscle glycogen levels are less sensitive to fasting and feeding cycles. Strenuous exercise can deplete muscle glycogen stores, which can be replenished by new synthesis
(affects roughly 70% of the adult population worldwide), can be congenital or adult onset
Lactose intolerance
inability to metabolize galactose, rare but severe genetic disorder
Galactosemia
includes both glycolysis and gluconeogenesis disorders
Disorders affecting glucose metabolism
•Glycogen storage diseases (GSD): impair glycogen synthesis, glycogen degradation or glycolysis (more then 15 different disorders)
•Glycogen storage diseases (GSD): impair glycogen synthesis, glycogen degradation or glycolysis (more then 15 different disorders)
impair glycogen synthesis, glycogen degradation or glycolysis (more then 15 different disorders)
Glycogen storage diseases (GSD)
Fructose malabsorption and Hereditary fructose intolerance
Genetic disorders associated with defects in carbohydrate metabolism
Glycogen Storage Disease
I – Von Gierke disease
Deficient Enzyme
Glucose-6-phosphatase
Glycogen Storage Disease
II – Pompe diseasea
Deficient Enzyme
Acid α-glucosidase
(acid maltase)
Glycogen Storage Disease
III – Cori diseasea
Deficient Enzyme
Glycogen debranching enzyme
(4:4 transferase)
Glycogen Storage Disease
IV – Andersen disease
Deficient Enzyme
Glycogen branching enzyme
(4:6 transferase)
Glycogen Storage Disease
V – McArdle diseasea
Deficient Enzyme
Muscle glycogen phosphorylase
(myophosphorylase)
Glycogen Storage Disease
VI – Hers disease
Deficient Enzyme
Liver glycogen phosphorylase
Glycogen Storage Disease
VII – Tarui disease
Deficient Enzyme
Muscle phosphofructokinase
Concept map for fructose and galactose metabolism
GALT = galactose 1-phosphate uridyltransferase
Fructose and Galactose metabolism
Fructose & Aldose B
Galactose & GALT
Glycosaminoglycans (GAGs) are complex heteropolysaccharide chains associated with proteoglycans to form a gel-like matrix, which is a component of the _____ in tissues
extracellular matrix (ECM)
forms mucous secretions (mucopolysaccharides) and synovial fluids, contributing to their viscous and lubricating properties
Glycosaminoglycans (GAGs)
The flexible nature of ________ allows for force absorption in cartilage, synovial fluids and the vitreous humor of the eye
glycosaminoglycans
The six major types of GAGs are divided according to monomeric composition, type of glycosidic linkages and location of sulfate units
components of extracellular matrix
composed of proteins and Glycosaminoglycans
Proteoglycans
Proteoglycan aggregate formation with ______ via non-covalent, ionic interactions
hyaluronic acid
A cartilage proteoglycan monomer can contain 100 linear chains consisting of up to 200 disaccharide units
The main protein family that contributes to cartilage formation is ________
aggrecan
joint cartilage is degraded and proteoglycans that normally help provide a cushion for the joints are lost
Osteoarthritis
glucosamine and chondrotin have been reported to both relieve pain and stop progression of _______
osteoarthritis
Deficiencies and imbalance of ______ is associated with a large variety of diseases, including atherosclerosis, diabetes and obesity
lipid metabolism
Carbon atoms are organic molecules comprised primarily of carbon, oxygen and hydrogen molecules. They are chemically defined as
polyhydroxy aldehydes, or ketones.
(CH2O)n
Includes sugars, alcohols and polysaccharides
Carbohydrates are classified based on the number of _____ that makes the molecule
carbons
Carbohydrates are found in nature in various forms, including:
Monomers: monosaccharides
Dimers: disaccharides
OligysaccharidesPolymers: polysaccharides
Glucose metabolite linked to diabetic complications and retinopathy
sorbitol
Note that Milk products are the best source for _____
Galactose
Monosaccharide’s with 5 or more carbons form cyclic structures when in
solution
Phospholipids
→phosphatidyl choline → surfectant → alveoli lungs
1.Phospholipids: Lipids containing, in addition to fatty acids and an alcohol, a phosphoric acid residue. They frequently have nitrogen-containing bases (e.g., choline). In many phospholipids the alcohol is glycerol (glycerophospholipids), but in sphingophospholipids it is sphingosine, which contains an amino group.
Glycolipids (glycosphingolipids): Lipids containing a fatty acid, sphingosine, and carbohydrate.
glucocerebrosides → brain
Other complex lipids: Lipids such as sulfo-lipids and amino-lipids. Lipoproteins may also be placed in this category.
Precursor and derived lipids: These include fatty acids, glycerol, steroids, other alcohols, fatty aldehydes, ketone bodies, hydrocarbons, lipid-soluble vitamins and micronutrients, and hormones.
_______ is one of the major phosphoacylglycerols found in membranes and serfectant
Phosphatidylcholine (lecithin)
If only a phosphate group is attached to carbon 3, the compound is _______Phosphatidic acid is a precursor for the synthesis of the other phosphoacylglycerols.
phosphatidic acid
Ether glycerophospholipids: Platelet-activating factor (PAF)
•Has a saturated alkyl group in an ether link to carbon 1 and an acetyl residue, rather than a tatty acid, at carbon 2 of the glycerol backbone•Activates inflammatory cells to mediate a wide range of inflammatory reactions including, thrombosis, hypersensitivity and anaphylaxis.•Induces platelet aggregation and activation of neutrophils and alveolar macrophages.
Phospholipids
When phospholipids are dispersed in aqueous solution, they spontaneously form lamellar structures, and under suitable conditions, they organize into bilayer vesicular structures termed liposomes.
Diseases associated with deficient sphingolipid metabolism (Sphingolipidosis)
Tay-sacs disease
Enzyme
Hexosaminidase A
Diseases associated with deficient sphingolipid metabolism (Sphingolipidosis)
Farbry disease
Enzyme
a- Galactosidase
Diseases associated with deficient sphingolipid metabolism (Sphingolipidosis)
Metachromatic Leukodystrophy
Emzyme
Arylsulfatase-A
Diseases associated with deficient sphingolipid metabolism (Sphingolipidosis)
Krabbe disease
Enzyme
B-Galatosidase
Diseases associated with deficient sphingolipid metabolism (Sphingolipidosis)
Gaucher disease
B-Glucosidase
Diseases associated with deficient sphingolipid metabolism (Sphingolipidosis)
Niemann Pick disease
Enzyme
Sphingomeylinase
Diseases associated with deficient sphingolipid metabolism (Sphingolipidosis)
Farber disease
Enzyme
Ceramidase
Sphingomyelin, which contains a phosphorylcholine group attached to ceramide, is a component of cell membranes and the myelin sheath around neurons.
electrical insulator in myelin sheath
Triacylglycerol (TAG)
Glycerol and 3 fatty acids. Function: Storage form of lipids (for energy)
Phosphatidylinositol
Glycerol fatty acids, inositol and phosphate. Function: Role in cell signaling
