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بسم الله الرحمن الرحيم
Glycolipids By Amr S. Moustafa, M.D.; Ph.D.
Outlines Overview and Functions Structure and TypesSynthesis and Degradation Sphingolipidosis
Overview Essential component of membranes Abundant in nervous tissueExtra-nervous tissue: e.g., Receptors for Cholera toxins Diphtheria toxins Viruses
Overview Regulation of growth & development Very antigenic:CONT’D Regulation of growth & development Very antigenic: Blood group antigen Embryonic antigen Tumor antigen Cell transformation
Sphingolipids: Structure and TypesCeramide = Sphingosine + fatty acid Sphingomyelin = Ceramide + Phosphorylcholine Cerebrosides = Ceramide + Monosaccharides Globoosides = Ceramide + Oligosaccharides Gangliosides = Ceramide oligosaccharides + NANA
Neutral GlycosphingolipidsCerebrosides = Ceramide monosaccharides Galactocerbrosides = Ceramide + Galactose Glucocerbrosides = Ceramide + Glucose Globosides = Ceramide Oilgosaccharides Lactosylceramide = Cer-Glc-Gal Forssman antigen = Cer-Glc-(Gal)2-(GalNac)2
Acidic GlycosphingolipidsNegatively charged at physiological pH Gangliosides: Most complex Ganglion cells Ceramide oligosaccharides + NANA 2. Sulfatides: Nerve tissue and kidneys Cerbrosides + Sulfated Gal
Sulfate donor: PAPS
Sulfatides Galactocerbroside 3-sulfate
Sphingolipids’ DegradationLysosomal hydrolytic enzymes Last on, First off Defective: Total or partial Sphingolipidosis
Sphingolipidosis Synthesis (Normal); Degradation (Defective)Substrate accumulates in organs Progressive, early death Phenotypic and genotypic variability Autosomal recessive, Except Fabry (X-linked) Rare, Except Ashkenazi Jewish
Sphingolipidosis Diagnosis: Treatment: CONT’D Measure enzyme activityCultured fibroblasts or peripheral leukocytes Cultured amniocytes (prenatal) Histologic examination DNA analysis Treatment: Replacement Therapy: Recombinant human enzyme Bone marrow transplantation: Gaucher disease
Niemann - Pick Disease
Prostaglandins Eicosanoids (20 C): Prostaglandins (PG)Thromboxanes (TX) Leukotrienes (LT) PG Vs Hormones: All cells Local acting & Not stored Short half-life Plasma membrane & nuclear receptors
Prostaglandin’S SynthesisDietary precursor: Linoleic acid 18:2 (9, 12) Immediate precursor: Arachidonic acid 20:4 (5, 8,11,14) Prostaglandin endoperoxide synthase: Microsomal enzyme Two Catalytic activities: Fatty acid cyclooxygenase (COX), 2 O2 Peroxidase (GSH) Parent Prostaglandin : PGH2
COX Isoenzymes COX-1: Constitutive Most tissues Important for:Integrity of gastric mucosa Renal homeostasis Platelet aggregation
COX Isoenzymes CONT’D COX-2 Inducible Limited No. of tissues Stimulus:Products of inflammatory & immune cells Mediates: Inflammatory response: Pain, Redness & Swelling Fever of infection
COX Inhibitors Cortisol (Steroid): Selective COX-2 (-)Phospholipase A2 (-) NSAIDS: Both COX-1 and –2 (-) Side effects: Gastric, Renal & Clotting Low-dose Asprin therapy Celecoxib: Selective COX-2 (-)
Complex Lipid Metabolism UNIT III: Lipid Metabolism.
Prostaglandins & Related compounds. EICOSANOIDS Compounds that originate from polyunstaurated fatty acids with 20 carbons Prostaglandins (PG) Prostaglandins.
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Prostaglandins& Related Compounds. Objectives Origin of ecosanoids Ecosanoids role Overview of the structure Role of phospholipase A2 Cyclooxgenase isoenzymes.
Complex Lipid Metabolism
Complex Lipids. Introduction: A 3 week premature baby boy born to a diabetic mother by cesarean section. Presenting complaints: Bluish discoloration of.
Metabolism of dietary lipids Biochemistry Department.
THEY ARE SUGAR CONTAINING LIPIDS. THEY ARE DERIVED FROM SPHINGOLIPDS. THEY DO NOT CONTAIN PHOSPHATE. GLYCOLIPIDS ARE WIDELY DISTRIBUTED IN OUR.
Prostaglandin, Leukotriene, and Thromboxane Synthesis Eric Niederhoffer SIU-SOM Linoleic acid Thromboxane A 2 synthase Lipoxygenase Arachidonic acid Prostaglandin.
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Prostaglandin, Leukotriene, and Thromboxane Synthesis Eric Niederhoffer SIU-SOM Pathway overview Prostaglandin receptors Pathway details Differential actions.
General, Organic, and Biological Chemistry Copyright © 2010 Pearson Education, Inc.1 Chapter 17 Lipids 17.6 Sphingolipids.
EICOSANOIDS (prostaglandins, thromboxanes, leukotrienes)
COMPLEX LIPID METABOLISM
Stearic acid (C18) Oleic acid fatty acids ALA Essential 3.
Biological Lipids and Bilayers Lipids are nearly insoluble in Water –Form Biological membranes –Regulate solute flow and signalling –Provide concentrated.
Fats. Fatty Acid Fatty acids exist “free” in the body Also found as fatty acyl esters such as triacylglycerol’s. Low levels of free fatty acids occur.
Sphingolipids C-C-C Pseudo glycerol N CH 3 CCCCCCCCCCCCCCC CCCCCCCCCCCCCCCC HO OH OPO 3 -CH 2 CH 2 N(CH 3 ) 3 Oligosaccharides Glycolipids Long Chain fatty.
Metabolism of Acylglycerols & Sphingolipids
Sphingolipids Dr. Abir Alghanouchi Biochemistry department Sciences college.
Taif University College of Medicine Preparatory Year Students
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Metabolism of acylglycerols and sphingolipids Alice Skoumalová.
Fatty Acids - Fatty acids R-COOH (R is a long hydrocarbon chain) are the major components of triacylglycerols, glycerophospholipids, and sphingolipids.
COO - CH 2 COO - CH 2 C COO - O AcCoA COO - CH 2 OC HCOO - CH 2 CH 3 C-SCoA O COO - CH 2 C COO - O CH 2 HC COO - OH COO - CH 3 C O CO2CO2 COO - CH 2 C.
Prostaglandins, cyclo-oxygenase and the GIT HPETE PGH.
GLCOLIPIDS(GLYCOSPHONGOLIPIDS) Ceramide+carbohydrate (glucose,galactose,oligosacchride side chain)=GLYCOLIPID Cerebrosides: contain a single moiety, principally.
The Cell-Derived Mediators of Chemical Mediators of Inflammation Presented by Sara M. Al-Shaker Wed. 5/11/2008 King Saud University Riyadh, KSA.
Leukodystrophies Costello, D. J., A. F. Eichler, and F. S. Eichler. "Leukodystrophies: Classification, Diagnosis, and Treatment." Neurologist 15, no. 6.
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Carbohydrates of Physiologic Significance CARBOHYDRATES ARE ALDEHYDE OR KETONE DERIVATIVES OF POLYHYDRIC ALCOHOLS (1) Monosaccharides are those carbohydrates.
Lipids “Fat, what good is it?”. Lipids Biomolecules soluble in organic solvents Roles –Nutritional –Structural –regulatory.
Lipid Compounds of Physiological Significance By Amr S. Moustafa, MD, PhD.
Do you administer NSAIDs or steroids to these patients?
ESSENTIALS OF GLYCOBIOLOGY
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