VI - Special Topics Flashcards
Long unbranched heteropolysaccharide chains composed of a repeating disaccharide unit
Glycosaminoglycans
Glycosaminoglycans: Amino Sugars
N-acetylglucosamine, N-acetylgalactosamine
Glycosaminoglycans: Acidic Sugars
Glucuronic Acid, Iduronic Acid
Glycosaminoglycans: (GlcNAc, GlcUA), synovial fluid, vitreous humor, loose connective tissue
Hyaluronic Acid
Glycosaminoglycans: (GalNAc, GlcUA), cartilage, bone, cornea
Chondroitin Sulfate
Glycosaminoglycans: (GlcNAc, Gal), cornea, loose connective tissue
Keratan Sulfate
Glycosaminoglycans: (GlcN, IdUA), mast cells
Heparin
Glycosaminoglycans: (GlcN, GlcUA), skin, aortic wall
Heparan Sulfate
Glycosaminoglycans: (GalNAc, IdUA), wide distribution
Dermatan Sulfate
All GAGs are covalently attached to proteins to form proteoglycans except
Hyaluronic Acid (occurs indipendently)
Covalently linked to roteins via a trihexoside serine O-glycosidic bond
Glycosaminoglycans
Structural components of the extracellular matrix, interacts with collagen, elastin, fibronectin, laminin and growth factors, bind polycations and cations as polyanions, turgor of various tissues, sieves in the ECM
Glycosaminoglycans
Glycosaminoglycans: important role in permitting cell migration during morphogenesis and wound repair, attracts water into the extracellular matrix, contributes to compressibility of cartilage
Hyaluronic Acid
Glycosaminoglycans: located at sites of calcification in endochondral bone and in cartilage
Chondroitin Sulfate
Glycosaminoglycans: play a critical role in corneal trarnsparency
Keratna Sulfate I and Dermatan Sulfate
Glycosaminoglycans: have a structural role in sclera
Dermatan Sulfate
Glycosaminoglycans: important anticoagulant, binds with factors IX and XI but its most important interaction is with plasma antithrombin III
Heparin
Glycosaminoglycans: component of plasma membranes where they may act as receptors and participate in cell adhesion and cell-cell interactions, determine charge selectiveness of renal glomerulus, component of synaptic and other vesicles
Heparan Sulfate
Synthesis of GAGs: polysaccharide chains are elongated by the sequential addition of alternating acidic and amino sugars donated by
UDP derivatives
Synthesis of GAGs: catalyzed by a family of specific
transferases
Synthesis of GAGs: Location
endoplasmic reticulum, Golgi apparatus
Degradation of GAGs: GAGs are phagocytosed, and in order to be degraded, the vesicles fuse with
lysosomes
Degradation of GAGs: lysosomal degradation of GAGs is accomplished by
acid hydrolases
Accumulation of GAGs in lysosomes due to deficiency in hydrolases
Mucoolysaccharidoses
Mucoolysaccharidoses: all are autosomal recessive except
Hunter’s Syndrome (X-linked Recessive)
Mucoolysaccharidoses: all present with mental retardation except
Morquio’s Syndrome
Mucoolysaccharidoses: Type IH, α-L-iduronidase deficiency, corneal clouding, cardiomyopathy, mental retardation
Hurler’s Syndrome
Mucoolysaccharidoses: Type II, iduronate sulfatase deficiency, no corneal clouding, cardiomyopathy, mental retardation
Hunter’s Syndrome
Mucoolysaccharidoses: Type IV, galactose-6-sulfatase deficiency, no CNS involvement, skeletal dysplasia, short stature
Morquio’s Syndrome
Proteins to which oligosaccharides are covalently attached
Glycoproteins
Short & branched carbohydrate chains, no repeating sugar units
Glycoproteins
Long & linear carbohydrate chains, repeating sugar units
Proteoglycans
Cell surface recognition, antigenicity, components of the extracellular matrix and of the mucins of the gastrointestinal and urogenital tracts
Glycoproteins
Major Classes of Glycoproteins
O-linked, N-linked, Glycosylphosphatidyinositol (GPI)-linked
O-linked Glycoproteins
Hydroxyl Groups: Serine, Threonine
N-linked Glycoprotein
Amide Group: Asparagine
Occurs through interaction of ZP3, an O-linked glycoprotein in the zona pellucida, with a surface protein on the sperm surface (galactosyl transferase)
Fertilization
Circulating leukocytes adhere to the endothelium through selectins on the latter’s cell surface
Inflammation
Deficient phosphorylation of mannose residues in N-linked glycoprotein pre-enzymes, defect in labelling of pre-enzymes so they can be correctly transported to lysosomes
I-cell Disease
Skeletal abnormalities, restricted joint movement, coarse facial features, severe psychomotor impairment
I-cell Disease
Glycoprotein of the influenza virus that allows new viruses to exit infected cells
Neuraminidase
HIV surface glycoprotein used to attached to cells
gp120
HIV surface glycoprotein used to fuse with the host cell membrane
gp41
Alteration in the glycosylation of circulating IgG molecules such that they lack galactose in their Fc regions and terminate in GlcNAc
Rheumatoid Arthritis
In Malaria, attachment of plasmodium falciparum to human cells is mediated by a _____ present on the surface of the parasite
GPI-linked Glycoprotein
Discovered by Landsteiner in 1900, membranes of the RBCs of most individuals contain one blood group substance
ABO Classification
On membranes of RBCs, the oligosaccharides that determine the specific natures of the ABO substances appear to be mostly present in
glycosphingolipids
Antigen on Type A blood
N-Acetylgalactosamine
Antigen on Type B blood
Galactose
Antigen on Type AB blood
N-Acetylgalactosamine, Galactose
Polypeptide hormones secreted by β-cells of the islets of Langerhans of the endocrine pancreas, stimulated by high blood glucose, inhibited by epinephrine
Insulin
Its receptor has tyrosine kinase activity leading to a cascade of cell-signalling responses, increased anabolism, decreased catabolism
Insulin
Mechanisms stimulated by Insulin
glucose uptake, synthesis of glycogen, protein and fat
Mechanisms inhibited by Insulin
glycogenolysis, gluconeogenesis, ketogenesis, lipolysis
Polypeptide hormone secreted by the α-cells of the islets of Langerhans of the endocrine pancreas, timulated by low blood glucose, AA and epinephrine, Inhibited by high blood glucose and insulin
Glucagon
Its receptor is a G-protein that activates adenylate cyclase to increase cAMP and protein kinase A, maintains blood glucose during periods of hypoglycemia
Glucagon
Mechanisms stimulated by Glucagon
gluconeogenesis, glycogenolysis, ketogenesis, uptake of AA
Mechanism inhibited by Glucagon
glycogenesis
Hormones that counteract insulin
glucagon, cortisol, growth hormone epinephrine
Hypoglycemia: Glucose Level
< 40 mg/dL
In ethanol intoxication, there will be less _____ & _____ hence there will be less substrates for _____ predisposing the body to _____.
OAA, pyruvate, gluconeogenesis, hypoglycemia
2-4 hour period after ingestion of a normal meal, transient increases in plasma glucose, AAs and TGs, increased insulin, decreased glucagon, anabolic
Absorptive/Fed State
Fed State: fuel for most tissues
glucose
Fed State: replenishes its glycogen stores, replaces any needed hepatic proteins, increased TG synthesis
liver
Fed State: increased TG synthesis and storage
adipose
Fed State: increased protein synthesis
muscle
Fed State: uses glucose exclusively as fuel
brain
In the abscence of food, plasma levels of glucose, AAs and TGs fall, decreased insulin, increased glucagon, catabolic
Fasting State
Fasting State: degrades glycogen, initiates gluconeogenesis, increased FA oxidation as a source of energy and to supply the acetyl CoA building blocks for ketone body synthesis
liver
Fasting State: degrades stored TG thus providing FA and glycerol to the liver
adipose
Fasting State: can also use FA as well as ketone bodies, protein is degraded to supply AA for gluconeogenesis in the liver
muscle
Fasting State: uses ketones in prolonged fasting
brain
Energy-rich molecules larger than that of the other dietary nutrients, fats, carbohydrates, protein, ethanol
Macronutrients
Nutrients needed in lesser amounts, vitamins and minerals
Micronutrients
Average daily nutrient intake level estimated to mee the requirement of 50% of healthy individuals in a particular life stage and sex
Estimated Average Requirement (EAR)
Average daily dietary intake level that is sufficient to meet the requirements of >95% of all individuals in a life stage and sex
Recommended Daily Allowance (RDA)
Arbitrarily set in the absence of scientific evidence to calculate an EAR or RDA
Adequate Intake
Highest average nutrient intake level that is likely to pose no risk of adverse health effects to almost all individuals in the general population
Tolerable Upper Intake Level
Average dietary energy intake predicted to maintain an energy balance in a healthy adult of a defined age, gender and height whose weight and level of physical activity are consistent with good health
Estimated Energy Requirement
Estimated Energy Requirement: Sedentary
30 kcal/kg/day
Estimated Energy Requirement: Moderately Active
35 kcal/kg/day
Estimated Energy Requirement: Very Active
40 kcal/kg/day
Total Caloric Requirement: In-Patients
35 kcal/kg/day
Energy Requirements: Fats
20-35%
Energy Requirements: Carbohydrates
45-65%
Energy Requirements: Proteins
10-35%
Energy Content: Fats
9 kcal/gram