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Understanding Ciliopathy: Insights into Alström Syndrome, Glucose Metabolism, and Organ Fibrosis

This article explores the microscopic structure and function of cilia in relation to Alström syndrome, a condition characterized by organ fibrosis and metabolic issues. It discusses the role of glucose transport, insulin sensitivity, and intermediary metabolism, highlighting how exercise and dietary modifications can improve health outcomes. We examine the effects of incretin hormones on glucose regulation and their implications for diabetes management. The interplay between ciliary function, intracellular trafficking, and metabolic pathways offers critical insights into potential therapeutic interventions.

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Understanding Ciliopathy: Insights into Alström Syndrome, Glucose Metabolism, and Organ Fibrosis

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  1. The cilium 1 microscopic structure NSCT

  2. The Cilium 2 intracellular trafficking NSCT

  3. photoreceptor Glucose transport Smooth muscle cilium cochlea

  4. Organ fibrosis in Alström syndrome NSCT

  5. Improvements with surgery https://weightloss.clevelandclinic.org/images/Reduction3.gif

  6. CARNITINE ACYL TRANSFERASE TCA CYCLE INSULIN EFFECTS ON INTERMEDIARY METABOLISM PLASMA HEPATOCYTE PLASMA LACTATE GLYCOGEN PYRUVATE PHOSPHORYLASE GLYCOGEN SYNTH రర GLUCONEOGENESIS GLUCOSE GLUCOKINASE FRUCTOSE 6 PHOS GLYCOLYSIS INSULIN SENSITIVE LIPOPROTEIN LIPASE GLUT RECEPTOR PYRUVATE VLDL CHOLESTEROL FA + GLYCEROL LDL MELONYL FATTY ACIDS HMG CoA ACETYL CoA CoA MEVALONIC ACID REDUCTASE TG HDL VLDL TRIGLYCERIDE KETONE BODIES ADIPOCYTE OXIDATIVE PHOSPHORYLATION 30ATP INSULIN SENSITIVE MITOCHONDRION

  7. Exercise and insulin sensitivity NSCT

  8. Effect of BMI on diabetes rate in Alström syndrome % NSCT

  9. Within family diabetes trend progression remission

  10. Ingestion of food • Glucose dependent • Insulin from beta cells(GLP-1 and GIP) Insulinincreases peripheral glucose uptake GI tract Pancreas Release of incretin gut hormones Beta cells Alpha cells Active GLP-1 and GIP Increased insulin and decreasedglucagon reduce hepatic glucose output • Glucagon from alpha cells (GLP-1)Glucose dependent Incretins and glycaemic control7,8 Bloodglucose control DPP-4enzyme rapidly degrades incretins NSCT Adapted from 7. Drucker DJ. Cell Metab. 2006;3:153–165. 8. Miller S, St Onge EL. Ann Pharmacother 2006;40:1336-1343.

  11. Absence of neuropathy in Alström type 2 diabetes NSCT

  12. Organ fibrosis in Alström syndrome Transplant, Valve dysfunction ?prevention, TIPS, partial transplant transplant NSCT

  13. Coronary artery disease Duplex-PVD Diabetes Renal dysfunction High lipids hypertension Carotid atheroma NSCT PVD

  14. What diet? What Exercise? Reduced carbohydrate Bespoke Weight training Horse riding Walking Swimming Cycling Dancing NSCT

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