Glycogen Metabolism.pptx
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1. Glycogen is the storage form of carbohydrate found in the liver and muscle in the form of granules. Glycogen synthesis (glycogenesis) and breakdown (glycogenolysis) are regulated by enzymes such as glycogen synthase and glycogen phosphorylase. 2. Disorders of glycogen metabolism can occur due to defects in glycogen branching enzymes or debranching enzymes. Common glycogen storage disorders include Von Gierke's disease, Pompe's disease, and McArdle's disease which are characterized by organ involvement and symptoms such as hypoglycemia, hepatomegaly, and muscle weakness. 3. Glycogen synthesis and breakdown are regulated by hormones,
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Final lect; 8 glycogen storage diseases
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Glycogen storage disease
Glycogen storage disease is a group of metabolic disorders caused by the body's inability to properly store glycogen. There are several types classified by the enzyme deficiency. Type I involves the liver and causes low blood sugar and high lactate levels. Type III is caused by a debranching enzyme deficiency and results in an enlarged liver and low blood sugar. Type IV is a branching enzyme deficiency leading to liver enlargement and cirrhosis. Treatment focuses on managing symptoms and in some cases enzyme replacement therapy.
GLYCOGEN STORAGE DISEASE , GSD , Von Gierke Disease
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2.GSD III
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GLYCOGEN STORAGE DISEASES
This document summarizes several glycogen storage diseases caused by deficiencies in enzymes involved in glycogen synthesis and breakdown. Key points include: glycogen storage diseases are inherited disorders characterized by abnormal glycogen deposition; deficiencies in enzymes like glucose-6-phosphatase and acid maltase can cause hypoglycemia, lactic acidosis, hyperlipidemia, and other issues; the organs and severity of symptoms vary depending on the specific enzyme deficiency.
Metabolism of glycogen
Glycogen is the major storage form of glucose found mainly in the liver and skeletal muscles. It is formed from glucose through glycogenesis and broken down into glucose through glycogenolysis. These pathways are regulated by enzymes like glycogen synthase and glycogen phosphorylase in response to hormones like insulin and glucagon to maintain blood glucose levels. Deficiencies in enzymes of glycogen metabolism can result in glycogen storage diseases characterized by hypoglycemia, hepatomegaly and other symptoms.
Glycogen storage disorders pathology
Glycogen storage disease is caused by deficiencies in enzymes involved in glycogen metabolism. There are several types classified based on the enzyme deficiency and clinical manifestations. Type 1 is von Gierke disease where deficiency of glucose-6-phosphatase causes hypoglycemia and liver abnormalities. Type 2 is Pompe disease caused by acid alpha-glucosidase deficiency leading to glycogen accumulation in muscles. Treatments depend on the type but may include dietary modifications, enzyme replacement therapy, or gene therapy.
HMP SHUNT.pptx
The document summarizes several metabolic pathways including the hexose monophosphate shunt (HMP shunt), glycogen metabolism, glycogen storage diseases, and gluconeogenesis. The HMP shunt produces pentose and NADPH which are needed for biosynthesis. It is less active in muscles and more active in leukocytes where NADPH is used to produce radicals. Glucose-6-phosphate dehydrogenase deficiency is an X-linked genetic disorder where a defective enzyme causes red blood cell breakdown. Glycogen is the stored form of carbohydrates and is broken down by enzymes into glucose for energy. Glycogen storage diseases occur when enzymes are missing, causing glycogen to build up abnormally. Gl
Glycogen Metabolism
Glycogen is the storage from of glucose. The metabolism of glycogen both as glycogenolysis, breakdown of glycogen, and glycogenesis, formation of glycogen along with their regulation is briefed in the slides.
Recommended
Final lect; 8 glycogen storage diseases
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Glycogen storage disease
Glycogen storage disease is a group of metabolic disorders caused by the body's inability to properly store glycogen. There are several types classified by the enzyme deficiency. Type I involves the liver and causes low blood sugar and high lactate levels. Type III is caused by a debranching enzyme deficiency and results in an enlarged liver and low blood sugar. Type IV is a branching enzyme deficiency leading to liver enlargement and cirrhosis. Treatment focuses on managing symptoms and in some cases enzyme replacement therapy.
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Detailed presentation about glycogen storage disease.
description about all types of GSDs like .
1. GSD I
2.GSD III
3. GSD IV
4. GSD VI
5. GSD IX
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This document summarizes several glycogen storage diseases caused by deficiencies in enzymes involved in glycogen synthesis and breakdown. Key points include: glycogen storage diseases are inherited disorders characterized by abnormal glycogen deposition; deficiencies in enzymes like glucose-6-phosphatase and acid maltase can cause hypoglycemia, lactic acidosis, hyperlipidemia, and other issues; the organs and severity of symptoms vary depending on the specific enzyme deficiency.
Metabolism of glycogen
Glycogen is the major storage form of glucose found mainly in the liver and skeletal muscles. It is formed from glucose through glycogenesis and broken down into glucose through glycogenolysis. These pathways are regulated by enzymes like glycogen synthase and glycogen phosphorylase in response to hormones like insulin and glucagon to maintain blood glucose levels. Deficiencies in enzymes of glycogen metabolism can result in glycogen storage diseases characterized by hypoglycemia, hepatomegaly and other symptoms.
Glycogen storage disorders pathology
Glycogen storage disease is caused by deficiencies in enzymes involved in glycogen metabolism. There are several types classified based on the enzyme deficiency and clinical manifestations. Type 1 is von Gierke disease where deficiency of glucose-6-phosphatase causes hypoglycemia and liver abnormalities. Type 2 is Pompe disease caused by acid alpha-glucosidase deficiency leading to glycogen accumulation in muscles. Treatments depend on the type but may include dietary modifications, enzyme replacement therapy, or gene therapy.
HMP SHUNT.pptx
The document summarizes several metabolic pathways including the hexose monophosphate shunt (HMP shunt), glycogen metabolism, glycogen storage diseases, and gluconeogenesis. The HMP shunt produces pentose and NADPH which are needed for biosynthesis. It is less active in muscles and more active in leukocytes where NADPH is used to produce radicals. Glucose-6-phosphate dehydrogenase deficiency is an X-linked genetic disorder where a defective enzyme causes red blood cell breakdown. Glycogen is the stored form of carbohydrates and is broken down by enzymes into glucose for energy. Glycogen storage diseases occur when enzymes are missing, causing glycogen to build up abnormally. Gl
Glycogen Metabolism
Glycogen is the storage from of glucose. The metabolism of glycogen both as glycogenolysis, breakdown of glycogen, and glycogenesis, formation of glycogen along with their regulation is briefed in the slides.
Glycogen storage diseases
Glycogen storage diseases are caused by defects in glycogen synthesis or breakdown. There are several types affecting the liver or muscle. Type I is caused by glucose-6-phosphatase deficiency leading to liver enlargement and low blood sugar during fasting. Type III involves a debranching enzyme defect causing swollen abdomen and muscle weakness. Type VI deficiency of liver phosphorylase causes hepatomegaly, hypoglycemia, and growth issues. Symptoms, diagnosis, and treatments vary depending on the specific enzyme deficiency and tissues involved.
CH-03. Glycogen metabolism.pptx
Glycogen is the storage form of glucose found mainly in the liver and muscle. Glycogen metabolism involves glycogenesis, the formation of glycogen from glucose, and glycogenolysis, the breakdown of glycogen into glucose. Glycogenesis occurs through three phases - activation of glucose, glycosyl transfer, and branching. Glycogenolysis produces glucose through the actions of phosphorylase and debranching enzymes. Defects in enzymes involved in glycogen synthesis or breakdown can cause glycogen storage diseases, including Von Gierke's disease caused by glucose-6-phosphatase deficiency.
Glycogen storage disease (gsd)
Glycogen storage disorders are a group of inherited metabolic disorders caused by deficiencies in enzymes involved in glycogen synthesis or breakdown. There are several types classified by the affected enzyme and tissue. Type I, Von Gierke's disease, is caused by glucose-6-phosphatase deficiency affecting the liver and kidneys, leading to organ enlargement, high lactate levels, and gout. Type II, Pompe's disease, results from acid maltase deficiency impacting many tissues including heart, liver, and muscles. Symptoms range from cardiac failure in infants to late-onset muscle weakness. Treatment may include enzyme replacement therapy or diet modification.
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This document discusses glycogen storage diseases, which are inherited disorders caused by deficiencies in enzymes involved in glycogen metabolism. It summarizes several types of glycogen storage diseases, categorized based on the organ(s) affected and the specific enzyme deficiency. Type I (von Gierke's disease) is the most common and is caused by glucose-6-phosphatase deficiency, affecting the liver, kidneys, and intestines. Type II (Pompe's disease) is caused by acid alpha-glucosidase deficiency and can range from an infantile to adult form. Type III (Cori or Forbes disease) involves glycogen debranching enzyme deficiency in the liver and/or muscle. The document provides details on
GLCOGEN STORAGE DISORDERS
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Amer glycogen storage disease
Glycogen storage disease (GSD) occurs due to defects in enzymes involved in glycogen synthesis or breakdown, leading to excess glycogen storage. There are 11 known types classified by the affected enzyme and tissue. Symptoms vary by type but can include hypoglycemia, liver and muscle involvement, and exercise intolerance. Treatment depends on type and may include dietary changes, enzyme replacement therapy, or liver transplantation. Prenatal testing is available for some types.
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This document discusses glycogen metabolism pathways. It describes that glycogen metabolism has two pathways: glycogenesis and glycogenolysis. Glycogenesis is the formation of glycogen in the liver and muscles, requiring ATP and UTP. Glycogenolysis is the degradation of stored glycogen in the liver and muscles by glycogen degrading enzymes. Glycogen storage diseases can occur due to deficiencies in the enzymes involved in glycogenesis or glycogenolysis.
Biochemistry
Glycogen storage disease is a metabolic disorder caused by enzyme deficiencies affecting glycogen synthesis, breakdown, or glucose breakdown in muscles and liver cells. It can be genetic or acquired. Genetic glycogen storage disease is caused by inherited errors in metabolism resulting in defective enzymes, while acquired glycogen storage disease in livestock is caused by castanospermine intoxication. Some examples of genetic glycogen storage diseases include Von Gierke's disease, Pompe's disease, Cori's disease, and Anderson disease. Polyglucosan body disease/amylopectinosis is an extremely rare hereditary metabolic disorder caused by absence of the enzyme amylo-1,4,1,6-transglucosidase, which
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The document discusses several topics related to carbohydrate metabolism:
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2. Glycogen metabolism - Glycogen is stored glucose. Glycogenesis is the synthesis of glycogen from glucose-1-phosphate. Glycogenolysis breaks down glycogen to glucose-1-phosphate via phosphorylase.
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This document discusses the pathogenesis and etiology of metabolic disorders, including disorders of carbohydrate and lipid metabolism. It covers several key points:
1. Metabolic disorders can be caused by genetic factors like enzymopathies, damage to membranes/receptors, endocrine dysfunction, and neural impairment.
2. Dietary and digestive issues as well as other organ dysfunction can also contribute to metabolic disorders.
3. Glucose regulation is maintained through a balance of insulin and counter-regulatory hormones like glucagon, with disorders resulting in hyperglycemia or hypoglycemia.
4. The two primary types of diabetes mellitus - type 1 and type 2 - differ in etiology and pathogenesis
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The document summarizes key aspects of the hexose monophosphate pathway (HMP pathway or pentose phosphate pathway). It describes the pathway's location in the cytosol and key tissues where it is active. The oxidative and non-oxidative phases of the pathway and their reactions are outlined. Regulation of the pathway by NADPH concentration is mentioned. The significance of the pathway is that it generates pentoses and NADPH, which is important for biosynthesis and antioxidant reactions. Deficiencies in the pathway can cause hemolytic anemia.
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Glycogen storage diseases are a group of metabolic disorders caused by enzyme deficiencies that affect glycogen synthesis or breakdown in the liver and/or muscles. The main symptoms include low blood sugar, liver enlargement, slow growth, muscle weakness, and breakdown of muscle fibers. Diagnosis involves biopsy, blood and urine tests, and MRI scans. There are several types of glycogen storage diseases categorized by the specific enzyme deficiency and accumulated metabolite. Treatment focuses on maintaining blood glucose levels through nasogastric infusion, medications to manage symptoms, dietary changes, and in severe cases, organ transplantation.
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Glycogen is the storage form of glucose in animals and is stored primarily in the liver and muscle. Glycogen storage diseases are a group of metabolic disorders caused by defects in enzymes involved in glycogen synthesis or breakdown. The diseases are characterized by abnormal glycogen accumulation in tissues. Von Gierke's disease (type I) results from a deficiency in glucose-6-phosphatase leading to fasting hypoglycemia, lactic acidosis, hyperlipidemia, and hyperuricemia. The diseases vary in their symptoms and tissues affected depending on the specific enzyme deficiency.
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Glycogen storage diseases
Glycogen storage diseases are caused by defects in glycogen synthesis or breakdown. There are several types affecting the liver or muscle. Type I is caused by glucose-6-phosphatase deficiency leading to liver enlargement and low blood sugar during fasting. Type III involves a debranching enzyme defect causing swollen abdomen and muscle weakness. Type VI deficiency of liver phosphorylase causes hepatomegaly, hypoglycemia, and growth issues. Symptoms, diagnosis, and treatments vary depending on the specific enzyme deficiency and tissues involved.
CH-03. Glycogen metabolism.pptx
Glycogen is the storage form of glucose found mainly in the liver and muscle. Glycogen metabolism involves glycogenesis, the formation of glycogen from glucose, and glycogenolysis, the breakdown of glycogen into glucose. Glycogenesis occurs through three phases - activation of glucose, glycosyl transfer, and branching. Glycogenolysis produces glucose through the actions of phosphorylase and debranching enzymes. Defects in enzymes involved in glycogen synthesis or breakdown can cause glycogen storage diseases, including Von Gierke's disease caused by glucose-6-phosphatase deficiency.
Glycogen storage disease (gsd)
Glycogen storage disorders are a group of inherited metabolic disorders caused by deficiencies in enzymes involved in glycogen synthesis or breakdown. There are several types classified by the affected enzyme and tissue. Type I, Von Gierke's disease, is caused by glucose-6-phosphatase deficiency affecting the liver and kidneys, leading to organ enlargement, high lactate levels, and gout. Type II, Pompe's disease, results from acid maltase deficiency impacting many tissues including heart, liver, and muscles. Symptoms range from cardiac failure in infants to late-onset muscle weakness. Treatment may include enzyme replacement therapy or diet modification.
Blood glucose homeostasis
This document discusses glucose homeostasis and diabetes mellitus. It defines glucose homeostasis as maintaining blood glucose levels and describes the hormonal regulation of insulin and glucagon in different nutritional states like fed, fasting, and starvation. It also discusses the different phases of glucose regulation including the fed state where glucose is used by tissues, and the fasting state where the liver produces glucose through gluconeogenesis. The document further defines and classifies diabetes mellitus, describing the differences between type 1 and type 2 diabetes.
Glycogen disorder disease
This document discusses glycogen storage diseases, which are inherited disorders caused by deficiencies in enzymes involved in glycogen metabolism. It summarizes several types of glycogen storage diseases, categorized based on the organ(s) affected and the specific enzyme deficiency. Type I (von Gierke's disease) is the most common and is caused by glucose-6-phosphatase deficiency, affecting the liver, kidneys, and intestines. Type II (Pompe's disease) is caused by acid alpha-glucosidase deficiency and can range from an infantile to adult form. Type III (Cori or Forbes disease) involves glycogen debranching enzyme deficiency in the liver and/or muscle. The document provides details on
GLCOGEN STORAGE DISORDERS
GSD are inherited disorders of glycogen metabolism caused by defects in glycogen degradation, glycolysis or glycogen synthesis. They can affect the liver, muscle or be generalized. GSD I is the most severe liver GSD affecting glycogen breakdown and gluconeogenesis. It presents in infancy with hypoglycemia, hepatomegaly and growth retardation. Treatment involves preventing hypoglycemia through frequent feeds and nocturnal gastric feeding. GSD III is caused by debranching enzyme deficiency and can present with liver or muscle involvement. Symptoms are similar to GSD I in childhood but improve with age, while myopathy often appears later in life.
Metabolism of carbohydrates
This document provides an overview of carbohydrate metabolism. It discusses the major pathways including glycolysis, the citric acid cycle, gluconeogenesis, glycogen metabolism, the hexose monophosphate shunt, and the roles of hormones. Glycolysis converts glucose to pyruvate with ATP production. The citric acid cycle further oxidizes carbohydrates, lipids, and proteins to generate more ATP. Gluconeogenesis produces glucose from non-carbohydrates. Glycogen is stored glucose that is synthesized and broken down as needed. The hexose monophosphate shunt produces NADPH and pentoses using glucose. Hormones like glucagon and insulin regulate carbohydrate metabolism.
Amer glycogen storage disease
Glycogen storage disease (GSD) occurs due to defects in enzymes involved in glycogen synthesis or breakdown, leading to excess glycogen storage. There are 11 known types classified by the affected enzyme and tissue. Symptoms vary by type but can include hypoglycemia, liver and muscle involvement, and exercise intolerance. Treatment depends on type and may include dietary changes, enzyme replacement therapy, or liver transplantation. Prenatal testing is available for some types.
Carbohydrate metabolism
glucose- 6-phosphatase
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Fructose -6- phosphate
1) Glycogen synthesis (glycogenesis) and breakdown (glycogenolysis) pathways in the liver and muscle. Key enzymes involved include glycogen synthase, glycogen phosphorylase and debranching enzyme.
Fructose-1,6-bisphosphatase
2) Regulation of glycogen metabolism by hormones like insulin and glucagon via covalent modification of glycogen synthase and phosphorylase.
Fructose-1,6-bisphosphate
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This document discusses glycogen metabolism pathways. It describes that glycogen metabolism has two pathways: glycogenesis and glycogenolysis. Glycogenesis is the formation of glycogen in the liver and muscles, requiring ATP and UTP. Glycogenolysis is the degradation of stored glycogen in the liver and muscles by glycogen degrading enzymes. Glycogen storage diseases can occur due to deficiencies in the enzymes involved in glycogenesis or glycogenolysis.
Biochemistry
Glycogen storage disease is a metabolic disorder caused by enzyme deficiencies affecting glycogen synthesis, breakdown, or glucose breakdown in muscles and liver cells. It can be genetic or acquired. Genetic glycogen storage disease is caused by inherited errors in metabolism resulting in defective enzymes, while acquired glycogen storage disease in livestock is caused by castanospermine intoxication. Some examples of genetic glycogen storage diseases include Von Gierke's disease, Pompe's disease, Cori's disease, and Anderson disease. Polyglucosan body disease/amylopectinosis is an extremely rare hereditary metabolic disorder caused by absence of the enzyme amylo-1,4,1,6-transglucosidase, which
Bio chemo ( gsd )
GSD is a rare inherited metabolic disorder caused by deficient or defective enzymes involved in glycogen metabolism. This leads to abnormal glycogen accumulation in tissues like the liver, heart and kidneys. There are several types of GSD based on the specific enzyme deficiency. Type I is characterized by fasting hypoglycemia, hyperlipidemia and hyperuricemia due to glucose-6-phosphatase deficiency. Type II involves alpha-glucosidase deficiency causing glycogen buildup in lysosomes. Type III features abnormal glycogen storage with short branches due to glycogen debranching enzyme deficiency.
Glycogen Storage Disease
PharmD
Glycogen storage disease
Pathophysiology of GSD
Nine types of GSD
Epidemeology and inheritance patterns
4.3 gluconeogenesis&glycogen&ppp.ppt
The document discusses several topics related to carbohydrate metabolism:
1. Gluconeogenesis - The synthesis of glucose from non-carbohydrate sources in the liver and kidneys, which is essential for maintaining blood glucose levels. Key enzymes bypass irreversible steps of glycolysis.
2. Glycogen metabolism - Glycogen is stored glucose. Glycogenesis is the synthesis of glycogen from glucose-1-phosphate. Glycogenolysis breaks down glycogen to glucose-1-phosphate via phosphorylase.
3. The pentose phosphate pathway - It occurs in the cytosol and provides NADPH and ribose-5-phosphate without generating ATP. Glucose-6-phosphate
Pathophis of carbohydrates and lipids metabolism
This document discusses the pathogenesis and etiology of metabolic disorders, including disorders of carbohydrate and lipid metabolism. It covers several key points:
1. Metabolic disorders can be caused by genetic factors like enzymopathies, damage to membranes/receptors, endocrine dysfunction, and neural impairment.
2. Dietary and digestive issues as well as other organ dysfunction can also contribute to metabolic disorders.
3. Glucose regulation is maintained through a balance of insulin and counter-regulatory hormones like glucagon, with disorders resulting in hyperglycemia or hypoglycemia.
4. The two primary types of diabetes mellitus - type 1 and type 2 - differ in etiology and pathogenesis
HMP Shunt
The document summarizes key aspects of the hexose monophosphate pathway (HMP pathway or pentose phosphate pathway). It describes the pathway's location in the cytosol and key tissues where it is active. The oxidative and non-oxidative phases of the pathway and their reactions are outlined. Regulation of the pathway by NADPH concentration is mentioned. The significance of the pathway is that it generates pentoses and NADPH, which is important for biosynthesis and antioxidant reactions. Deficiencies in the pathway can cause hemolytic anemia.
GLYCOGEN STORAG-WPS Office.pptx
Glycogen storage diseases are a group of metabolic disorders caused by enzyme deficiencies that affect glycogen synthesis or breakdown in the liver and/or muscles. The main symptoms include low blood sugar, liver enlargement, slow growth, muscle weakness, and breakdown of muscle fibers. Diagnosis involves biopsy, blood and urine tests, and MRI scans. There are several types of glycogen storage diseases categorized by the specific enzyme deficiency and accumulated metabolite. Treatment focuses on maintaining blood glucose levels through nasogastric infusion, medications to manage symptoms, dietary changes, and in severe cases, organ transplantation.
Diabetes & gluconeogenesis
This document summarizes diabetes mellitus and the gluconeogenesis process. It discusses that diabetes is a metabolic disorder where the body cannot properly use or produce insulin, resulting in high blood glucose. The two main types of diabetes are described. Gluconeogenesis is introduced as the process where glucose is synthesized from non-carbohydrate sources like amino acids, particularly in diabetes where insulin is insufficient or cells are resistant to it. Key enzymes and regulatory steps in gluconeogenesis are outlined, noting its importance in providing glucose during periods of biological starvation in diabetes.
GLYCOGEN, GLYCOGEN METABOLIC DISEASE.
Glycogen is the storage form of glucose in animals and is stored primarily in the liver and muscle. Glycogen storage diseases are a group of metabolic disorders caused by defects in enzymes involved in glycogen synthesis or breakdown. The diseases are characterized by abnormal glycogen accumulation in tissues. Von Gierke's disease (type I) results from a deficiency in glucose-6-phosphatase leading to fasting hypoglycemia, lactic acidosis, hyperlipidemia, and hyperuricemia. The diseases vary in their symptoms and tissues affected depending on the specific enzyme deficiency.
Metabolic homeostasis
Metabolic homeostasis involves balancing catabolic and anabolic processes to maintain internal stability and allow growth. The AMP-dependent protein kinase (AMPK) regulates metabolic homeostasis by activating catabolism to produce ATP during low energy states and inhibiting anabolism to conserve ATP. Hormones like adiponectin, leptin, and ghrelin also control metabolic homeostasis by regulating AMPK activity and appetite. Disruptions to metabolic homeostasis can lead to diseases like diabetes mellitus.
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Glycogen Metabolism.pptx
- 1. GLYCOGEN METABOLISM & DISORDERS DR. NHETAN N. ACHARYA
- 2. Glycogen : storage form of carbohydrate. Organs: Liver & muscle. Organelle: Cytoplasm(present in granular form in cytosol). Rate limiting enzyme: Glycogen Synthase. Key Steps: - Synthesis of UDP glucose - Glycogen synthase reactions - Branching. Glycogen synthesized on a primer – Glycogenin (polypeptide). GLYCOGENESI S
- 3. GLYCOGENESI S • Synthesis of glycogen from glucose. 1. Synthesis of UDP Glucose 2. Glycogen Synthase Reactions 3. Formation of Branches in Glycogen.
- 4. Glycogen Synthase: rate limiting enzyme. In muscle & Liver Glycogen is stored is stored as 𝛃 particle. One 𝛃 particle – 60,000 glycogen residues. In liver : arranged in rosette pattern.
- 5. GLYCOGENOLYSIS It is the degradation of stored Glycogen in muscles and liver. Organelle: Cytoplasm, Lysosomes Rate limiting enzyme: Glycogen phosphorylase. Coenzyme: Pyridoxal Phosphate Steps: - Action of Glycogen Phosphorylase - Removal of Branches. - Conversion of Glucose-1 PO4 to Glucose.
- 8. • Debranching enzymes : ∝ 1,4 1,4 Glucan Transferase ∝ 1,6 Glucosidase/ Amylo 1, 6 glucosidase.
- 9. REGULATION OF GLYCOGENOLYSIS • Important in the maintenance of blood glucose levels. • 2 Most important Enzymes involved ( active forms) - Glycogen Synthase (De-phosphorylated state) - Glycogen Phosphorylase (Phosphorylated state) • Regulation of these enzymes is achieved through - Allosteric Regulation - Hormonal Regulation - Influence of Calcium
- 10. ALLOSTERIC REGULATION • Glycogen synthesis increases when available substrate (glucose) and energy levels are HIGH. The same goes for the opposite condition as well.
- 14. GLYCOGEN STORAGE DISORDERS TYPE 1- VON GIERKE’S DISEASE Defect: Glucose 6-phosphatase. Organs Involved: Liver, kidney & intestine. C/F: Hepatomegaly and increased renal size due to accumulation Hyperlipidemia Fasting hypoglycemia Ketosis Gouty Arthritis Lactic Acidemia.
- 15. TYPE II- POMPE’S DISEASE Defect: Lysosomal ∝ 1,4 glucosidase (acid maltase) Organs involved: all C/F: Cardiomegaly muscle weakness Hepatomegaly Prognosis is usually poor as death happens generally due to cardiac failure.
- 16. • TYPE III- CORI’S DISEASE (FORBE’ DISEASE) Defect: Amylo ∝ 1,6- glucosidase (Debranching enzyme) Organs involved: liver, muscle, heart, leucocytes C/F: similar symptoms but milder than VON GIERKE’S disease.
- 17. • TYPE IV- ANDERSON’S DISEASE Defect: Glucosyl 4-6 transferase Organs involved: most tissues C/F: hepatic accumulation resulting in impaired liver function.
- 18. • TYPE V- McArdle’s DISEASE Defect: Muscle Glycogen Phosphorylase. Organs involved: Skeletal Muscles. C/F: Unable to perform strenuous exercises. Suffer from muscle cramps and easy fatiguability. blood lactate and pyruvate level do not increase after exercise.
- 19. • TYPE VI- HER’S DISEASE Defect: Liver glycogen phosphorylase Organ involved: Liver C/F: Hepatomegaly Hypoglycemia & Ketosis seen. Moderate to good prognosis reported. Type VIII, IX, X and XI have been identified with similar enzyme defect
- 20. TYPE VII- TARUI’S DISEASE Defect: Phosphofructokinase Organ involved: Skeletal Muscle; erythrocytes C/F: Muscle cramps elicited in exercise Blood lactate levels NOT elevated Hemolysis can occur.
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