Disorders associated with fatty acid catabolism are caused by deficiencies in enzymes needed to break down fatty acids. This results in the buildup of fatty acid breakdown products and prevents the body from efficiently producing and using energy from fats. Specific disorders include medium-chain acyl-CoA dehydrogenase deficiency and carnitine uptake defect. Symptoms can include hypoglycemia, seizures, and delayed development. Treatment focuses on providing alternative energy sources and supplements.
25.1Digestion and Absorption of Lipids
25.2Triacylglycerol Storage and Mobilization
25.3 Glycerol Metabolism
25.4 Oxidation of Fatty Acids
25.5 ATP Production from Fatty Acid Oxidation
25.6 Ketone Bodies
25.7 Biosynthesis of Fatty Acids: Lipogenesis
25.8 Relationship Between Lipogenesis and Citric Acid Cycle Intermediates
25.9 Fate of Fatty-Acid Generated Acetyl CoA
25.10 Relationships Between Lipid and Carbohydrate Metabolism
25.11B Vitamins and Lipid Metabolism
1. Introduction to biochemistry: Cell and its biochemical organization, transport process across the cell membranes. Energy rich compounds: ATP, Cyclic AMP and their biological significance.
2. Biological oxidation: Coenzyme system involved in Biological oxidation. Electron transport chain (its mechanism in energy capture: regulation and inhibition): Uncouplers of ETC: Oxidative phosphorylation.
3. Enzymes: Definition: Nomenclature, IUB classification, Factor affecting enzyme activity, Enzyme action, enzyme inhibition. Isoenzymes and their therapeutic and diagnostic applications, Coenzymes and their biochemical role and deficiency diseases.
4. Carbohydrate metabolism: Glycolysis, Citric acid cycle (TCA cycle), HMP shunt, Glycogenolysis, gluconeogenesis, glycogenesis. Metabolic disorders of carbohydrate metabolism (diabetes mellitus and glycogen storage diseases): Glucose, Galactose tolerance test and their significance, hormonal regulation of carbohydrate metabolism.
5. Lipid metabolism: Oxidation of saturated (-oxidation): Ketogenesis and ketolysis, biosynthesis of fatty acids, lipids, metabolism of cholesterol, Hormonal regulation of lipid metabolism. Defective metabolism of lipids (Atherosclerosis, fatty liver, hypercholesterolemia).
6. Protein and amino acid metabolism: protein turn over, nitrogen balance, Catabolism of Amino acids (Transamination, deamination & decarboxylation).Urea cycle and its metabolic disorders, production of bile pigments, hyperbilirubinemia, porphoria, jaundice. Metabolic disorder of Amino acids.
7. Nucleic acid metabolism: Metabolism of purine and pyrimidine nucleotides, Protein synthesis, inhibition of protein synthesis
8. Introduction to clinical chemistry:
a) Urine analysis (macroscopic and physical examination, quantitative and
semi quantitative tests).
b) Test for NPN constituents. (Creatinine /urea clearance, determination of
blood and urine creatinine, urea and uric acid).
c) Test for hepatic dysfunction-Bile pigments metabolism.
d) Test for hepatic function: test- Serum bilirubin, urine bilirubin and urine
urobilinogen.
e) Lipid profile tests: Lipoproteins, composition, functions. Determination of
serum lipids, total cholesterol, HDL cholesterol, LDL cholesterol and
triglycerides.
25.1Digestion and Absorption of Lipids
25.2Triacylglycerol Storage and Mobilization
25.3 Glycerol Metabolism
25.4 Oxidation of Fatty Acids
25.5 ATP Production from Fatty Acid Oxidation
25.6 Ketone Bodies
25.7 Biosynthesis of Fatty Acids: Lipogenesis
25.8 Relationship Between Lipogenesis and Citric Acid Cycle Intermediates
25.9 Fate of Fatty-Acid Generated Acetyl CoA
25.10 Relationships Between Lipid and Carbohydrate Metabolism
25.11B Vitamins and Lipid Metabolism
1. Introduction to biochemistry: Cell and its biochemical organization, transport process across the cell membranes. Energy rich compounds: ATP, Cyclic AMP and their biological significance.
2. Biological oxidation: Coenzyme system involved in Biological oxidation. Electron transport chain (its mechanism in energy capture: regulation and inhibition): Uncouplers of ETC: Oxidative phosphorylation.
3. Enzymes: Definition: Nomenclature, IUB classification, Factor affecting enzyme activity, Enzyme action, enzyme inhibition. Isoenzymes and their therapeutic and diagnostic applications, Coenzymes and their biochemical role and deficiency diseases.
4. Carbohydrate metabolism: Glycolysis, Citric acid cycle (TCA cycle), HMP shunt, Glycogenolysis, gluconeogenesis, glycogenesis. Metabolic disorders of carbohydrate metabolism (diabetes mellitus and glycogen storage diseases): Glucose, Galactose tolerance test and their significance, hormonal regulation of carbohydrate metabolism.
5. Lipid metabolism: Oxidation of saturated (-oxidation): Ketogenesis and ketolysis, biosynthesis of fatty acids, lipids, metabolism of cholesterol, Hormonal regulation of lipid metabolism. Defective metabolism of lipids (Atherosclerosis, fatty liver, hypercholesterolemia).
6. Protein and amino acid metabolism: protein turn over, nitrogen balance, Catabolism of Amino acids (Transamination, deamination & decarboxylation).Urea cycle and its metabolic disorders, production of bile pigments, hyperbilirubinemia, porphoria, jaundice. Metabolic disorder of Amino acids.
7. Nucleic acid metabolism: Metabolism of purine and pyrimidine nucleotides, Protein synthesis, inhibition of protein synthesis
8. Introduction to clinical chemistry:
a) Urine analysis (macroscopic and physical examination, quantitative and
semi quantitative tests).
b) Test for NPN constituents. (Creatinine /urea clearance, determination of
blood and urine creatinine, urea and uric acid).
c) Test for hepatic dysfunction-Bile pigments metabolism.
d) Test for hepatic function: test- Serum bilirubin, urine bilirubin and urine
urobilinogen.
e) Lipid profile tests: Lipoproteins, composition, functions. Determination of
serum lipids, total cholesterol, HDL cholesterol, LDL cholesterol and
triglycerides.
The digestion of certain fats begins in the mouth, where short-chain lipids break down into diglycerides because of lingual lipase. The fat present in the small intestine stimulates the release of lipase from the pancreas, and bile from the liver enables the breakdown of fats into fatty acids.
Fatty acids can be liberated by simple hydrolysis of the ester bonds in triglycerides, but the insolubility of the triglycerides presents a problem; digestion occurs following dispersion of dietary fat into small particles with sufficiently exposed surface area for rapid attack by digestive enzymes. This is achieved by detergent action and mechanical mixing, with the detergent effect being supplied by several components, both in the diet and in the digestive juices, but especially by partially digested fats (fatty acid soaps and monacylglycerols) and by bile salts. (Cholic Acid)
Beta oxidation is the process of synthesis of energy molecules in the form of ATP from fathy acids. by this pathway much energy is produce as compare to proteins and charbohydrates. this is mitochondrial pathway as enzymes are present within mitochondria
Fatty Acids are Aliphatic carboxylic acids and each animal species will have characteristic pattern of fatty acid composition. Thus, human body fat contains 50% oleic acid, 25% palmitic acid, 10% linoleic acid and 5% stearic acid.
The digestion of certain fats begins in the mouth, where short-chain lipids break down into diglycerides because of lingual lipase. The fat present in the small intestine stimulates the release of lipase from the pancreas, and bile from the liver enables the breakdown of fats into fatty acids.
Fatty acids can be liberated by simple hydrolysis of the ester bonds in triglycerides, but the insolubility of the triglycerides presents a problem; digestion occurs following dispersion of dietary fat into small particles with sufficiently exposed surface area for rapid attack by digestive enzymes. This is achieved by detergent action and mechanical mixing, with the detergent effect being supplied by several components, both in the diet and in the digestive juices, but especially by partially digested fats (fatty acid soaps and monacylglycerols) and by bile salts. (Cholic Acid)
Beta oxidation is the process of synthesis of energy molecules in the form of ATP from fathy acids. by this pathway much energy is produce as compare to proteins and charbohydrates. this is mitochondrial pathway as enzymes are present within mitochondria
Fatty Acids are Aliphatic carboxylic acids and each animal species will have characteristic pattern of fatty acid composition. Thus, human body fat contains 50% oleic acid, 25% palmitic acid, 10% linoleic acid and 5% stearic acid.
Lipid metabolism is the synthesis and degradation of lipids in cells.
It involves the breakdown or storage of fats for energy and the synthesis of structural and functional lipids, such as those involved in the construction of cell membranes.
In animals, these fats are obtained from food or synthesized by the liver.
Ketone bodies, or simply ketones are substances produced by the liver during gluconeogenesis, a process which creates glucose in times of fasting and starvation. There are three ketone bodies produced by the liver. They are acetoacetate, beta-hydroxybutyrate, and acetone. These compounds are used in healthy individuals to provide energy to the cells of the body when glucose is low or absent in the diet.
A brief information about the SCOP protein database used in bioinformatics.
The Structural Classification of Proteins (SCOP) database is a comprehensive and authoritative resource for the structural and evolutionary relationships of proteins. It provides a detailed and curated classification of protein structures, grouping them into families, superfamilies, and folds based on their structural and sequence similarities.
Nutraceutical market, scope and growth: Herbal drug technologyLokesh Patil
As consumer awareness of health and wellness rises, the nutraceutical market—which includes goods like functional meals, drinks, and dietary supplements that provide health advantages beyond basic nutrition—is growing significantly. As healthcare expenses rise, the population ages, and people want natural and preventative health solutions more and more, this industry is increasing quickly. Further driving market expansion are product formulation innovations and the use of cutting-edge technology for customized nutrition. With its worldwide reach, the nutraceutical industry is expected to keep growing and provide significant chances for research and investment in a number of categories, including vitamins, minerals, probiotics, and herbal supplements.
(May 29th, 2024) Advancements in Intravital Microscopy- Insights for Preclini...Scintica Instrumentation
Intravital microscopy (IVM) is a powerful tool utilized to study cellular behavior over time and space in vivo. Much of our understanding of cell biology has been accomplished using various in vitro and ex vivo methods; however, these studies do not necessarily reflect the natural dynamics of biological processes. Unlike traditional cell culture or fixed tissue imaging, IVM allows for the ultra-fast high-resolution imaging of cellular processes over time and space and were studied in its natural environment. Real-time visualization of biological processes in the context of an intact organism helps maintain physiological relevance and provide insights into the progression of disease, response to treatments or developmental processes.
In this webinar we give an overview of advanced applications of the IVM system in preclinical research. IVIM technology is a provider of all-in-one intravital microscopy systems and solutions optimized for in vivo imaging of live animal models at sub-micron resolution. The system’s unique features and user-friendly software enables researchers to probe fast dynamic biological processes such as immune cell tracking, cell-cell interaction as well as vascularization and tumor metastasis with exceptional detail. This webinar will also give an overview of IVM being utilized in drug development, offering a view into the intricate interaction between drugs/nanoparticles and tissues in vivo and allows for the evaluation of therapeutic intervention in a variety of tissues and organs. This interdisciplinary collaboration continues to drive the advancements of novel therapeutic strategies.
Cancer cell metabolism: special Reference to Lactate PathwayAADYARAJPANDEY1
Normal Cell Metabolism:
Cellular respiration describes the series of steps that cells use to break down sugar and other chemicals to get the energy we need to function.
Energy is stored in the bonds of glucose and when glucose is broken down, much of that energy is released.
Cell utilize energy in the form of ATP.
The first step of respiration is called glycolysis. In a series of steps, glycolysis breaks glucose into two smaller molecules - a chemical called pyruvate. A small amount of ATP is formed during this process.
Most healthy cells continue the breakdown in a second process, called the Kreb's cycle. The Kreb's cycle allows cells to “burn” the pyruvates made in glycolysis to get more ATP.
The last step in the breakdown of glucose is called oxidative phosphorylation (Ox-Phos).
It takes place in specialized cell structures called mitochondria. This process produces a large amount of ATP. Importantly, cells need oxygen to complete oxidative phosphorylation.
If a cell completes only glycolysis, only 2 molecules of ATP are made per glucose. However, if the cell completes the entire respiration process (glycolysis - Kreb's - oxidative phosphorylation), about 36 molecules of ATP are created, giving it much more energy to use.
IN CANCER CELL:
Unlike healthy cells that "burn" the entire molecule of sugar to capture a large amount of energy as ATP, cancer cells are wasteful.
Cancer cells only partially break down sugar molecules. They overuse the first step of respiration, glycolysis. They frequently do not complete the second step, oxidative phosphorylation.
This results in only 2 molecules of ATP per each glucose molecule instead of the 36 or so ATPs healthy cells gain. As a result, cancer cells need to use a lot more sugar molecules to get enough energy to survive.
Unlike healthy cells that "burn" the entire molecule of sugar to capture a large amount of energy as ATP, cancer cells are wasteful.
Cancer cells only partially break down sugar molecules. They overuse the first step of respiration, glycolysis. They frequently do not complete the second step, oxidative phosphorylation.
This results in only 2 molecules of ATP per each glucose molecule instead of the 36 or so ATPs healthy cells gain. As a result, cancer cells need to use a lot more sugar molecules to get enough energy to survive.
introduction to WARBERG PHENOMENA:
WARBURG EFFECT Usually, cancer cells are highly glycolytic (glucose addiction) and take up more glucose than do normal cells from outside.
Otto Heinrich Warburg (; 8 October 1883 – 1 August 1970) In 1931 was awarded the Nobel Prize in Physiology for his "discovery of the nature and mode of action of the respiratory enzyme.
WARNBURG EFFECT : cancer cells under aerobic (well-oxygenated) conditions to metabolize glucose to lactate (aerobic glycolysis) is known as the Warburg effect. Warburg made the observation that tumor slices consume glucose and secrete lactate at a higher rate than normal tissues.
Richard's aventures in two entangled wonderlandsRichard Gill
Since the loophole-free Bell experiments of 2020 and the Nobel prizes in physics of 2022, critics of Bell's work have retreated to the fortress of super-determinism. Now, super-determinism is a derogatory word - it just means "determinism". Palmer, Hance and Hossenfelder argue that quantum mechanics and determinism are not incompatible, using a sophisticated mathematical construction based on a subtle thinning of allowed states and measurements in quantum mechanics, such that what is left appears to make Bell's argument fail, without altering the empirical predictions of quantum mechanics. I think however that it is a smoke screen, and the slogan "lost in math" comes to my mind. I will discuss some other recent disproofs of Bell's theorem using the language of causality based on causal graphs. Causal thinking is also central to law and justice. I will mention surprising connections to my work on serial killer nurse cases, in particular the Dutch case of Lucia de Berk and the current UK case of Lucy Letby.
This presentation explores a brief idea about the structural and functional attributes of nucleotides, the structure and function of genetic materials along with the impact of UV rays and pH upon them.
This pdf is about the Schizophrenia.
For more details visit on YouTube; @SELF-EXPLANATORY;
https://www.youtube.com/channel/UCAiarMZDNhe1A3Rnpr_WkzA/videos
Thanks...!
2. • Metabolism
enzymatic reactions through which the body derives energy and synthesizes essential molecules from fats, carbohydrates
and proteins we consume in food (Mandal, 2019), this can be broken down into two distinct processes:
i. Anabolism: the synthesis of all compounds needed by cells
ii. Catabolism: the breakdown of molecules to obtain energy
• Catabolic reactions
primarily oxidation reactions; reaction pathways include Glycolysis, the Citric Acid Cycle and the Electron Transport Chain
• Disease
Pathophysiological response to internal or external factors
• Disorder
A destruction to regular bodily structure and function
• Lipids
class of macromolecules that are non-polar and hydrophobic
includes fats, oils, waxes, phospholipids and steroids
Key Terms
3. • Fats (Triglycerides)
stored form of energy
made from fatty acids and glycerol
• Fatty acids
Long chain hydrocarbons with a carboxylic acid group at one end which can undergo esterification (reaction of an acid with
an alcohol in the presence of a catalyst) to become a lipid
Key Terms
Figure 1.0 showing formation of a triglyceride molecule (Symth, 2017)
4. Fatty Acid Catabolism
Fats (Triglycerides) are the most abundant dietary lipids whose function is the storage of
energy, it consists of a glycerol backbone that is esterified with three fatty acids (see
previous slide for visual).
When the body needs to use the energy stored as triglycerides it accesses it through a
mechanism known as the 𝛃-oxidation pathway (fatty acid catabolism).
This 𝛃-oxidation pathway occurs at 𝛃 carbon and produces acetyl CoA (further used in the
Citric Acid cycle for energy production) as the end product. This process occurs in the
peroxisome and mitochondria of cells (Vishwanath, 2016).
6. Fatty Acid Oxidation Disorder
There are 5 disorders associated with Fatty Acid Oxidation (Vishwanath, 2016):
1. Disorders of plasma membrane functions Carnitine uptake defect Long-chain
fatty acid transport/binding defect
2. Disorders of fatty acid transport across the mitochondrial membranes
3. Disorders of long-chain fatty acid β-oxidation
4. Disorders of medium-chain fatty acid β-oxidation: MCAD Deficiency
5. Disorders of short-chain fatty acid β-oxidation: SCAD Deficiency
7. Disorders of plasma membrane functions Carnitine uptake defect Long-chain fatty
acid transport/binding defect
● This is a fatty acid oxidation disorder that affects how the body uses fat as
energy
● It occurs as a result of the malfunction or non existence of the enzyme
carnitine transporter
● This enzyme functions to carry carnitine into the cells.
● Carnitine does the following:
● aids in the production of energy from the fat obtained in foods
● aids in the use of energy obtained from fat found in the body.
8. ● Symptoms that can occur are:
Sleepiness
Behavioral changes
Irrational mood
Poor appetite
● Treatment:
Dextrose is immediately administered through the vein to make up for the lack
of energy, supplements such as riboflavin, vitamin B2, may also be helpful
(Dr. Demczko, 2018).
Disorders of plasma membrane functions Carnitine uptake defect Long-chain fatty
acid transport/binding defect
9. ● This is a disorder of metabolism that is inherited.
● Can lead to K304E mutation of the ACADM gene where there is a change from
lysine to glutamate in mature MCAD proteins
● The lack of the enzyme acyl-CoA dehydrogenase leaves the body with inefficient
energy and allows breakdown products, such as acyl-CoA ,to accumulate
● Short chain and Long chain acyl-CoA dehydrogenase (MCAD) deficiency are
similar to medium chain acyl-CoA dehydrogenase (MCAD) deficiency, however
MCAD is the most common deficiency of the three
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency
10. Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency
● Symptoms that can occur are:
hypoglycemia due to drop in glucose level.
causes confusion or coma
vomiting or seizures
enlarged liver and enlarged heart muscle (resulting in irregular heartbeat)
● Treatment:
Dextrose is immediately administered through the vein to make up for the lack of
energy, supplements of the amino acid Carnitine may also be helpful (Dr.
Demczko, 2018).
11. Conclusion
Disorders Associated with Fatty Acid Catabolism are fatty acid oxidation
disorders which are caused by a lack of, or a deficiency in, enzymes
needed to break down fatty acids; this results in delayed mental and
physical development, especially in young children
12. References
Ahern, K., Rajagopal, I. (2019). Fatty Acid Oxidation. Oregon State University. Retrieved from
https://bio.libretexts.org/Bookshelves/Biochemistry/Book%3A_Biochemistry_Free_and_Easy_(Ahern_and_Rajagopal
)/06%3A_Metabolism_I/6.11%3A_Fatty_Acid_Oxidation
Dr. Demczko, M. (2018). Sidney Kimmel Medical Collge of Thomas Jefferson University. Retrieved from
https://www.msdmanuals.com/home/children-s-health-issues/hereditary-metabolic-disorders/fatty-acid-oxidation-
disorders
Dr. Mandal, A. (2019). What is Metabolism?. Retrieved from https://www.news-medical.net/life-sciences/What-is-
Metabolism.aspx
Ekinci, D. (2012). Biochemistry. Published by InTech Janeza Trdine 9, 51000 Rijeka, Croatia. ISBN 978-953-51-0076-8
Smyth, M. (2017). Biological Molecules: Lipids. Retrieved from https://tlamjs.com/2017/01/23/biological-molecules-lipids/
Vishwanath, V. A. (2016). Fatty Acid Beta-Oxidation Disorders: A Brief Review. Retrieved from
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4934411/
