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Related Concept Videos

Lipid Catabolism01:25

Lipid Catabolism

Triglycerides serve as crucial long-term energy storage molecules in microorganisms, providing a dense source of metabolic energy. Their breakdown is mediated by lipases, which hydrolyze triglycerides into glycerol and free fatty acids. Each of these components follows distinct metabolic pathways, ultimately contributing to ATP synthesis and cellular energy homeostasis.Glycerol MetabolismGlycerol, released from triglyceride hydrolysis, is phosphorylated by glycerol kinase to form...
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Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
Amino Acid Catabolism01:18

Amino Acid Catabolism

Microorganisms rely on proteins as an essential carbon and energy source, particularly in environments with limited polysaccharides or lipids. However, proteins are too large to cross the plasma membrane unaided, necessitating enzymatic degradation. Microbes secrete extracellular proteases and peptidases that hydrolyze proteins into peptides, which can then be transported across the membrane. Once inside the cell, intracellular proteases degrade these peptides into free amino acids, which...
The Electron Transport Chain01:30

The Electron Transport Chain

The electron transport chain or oxidative phosphorylation is an exothermic process in which free energy released during electron transfer reactions is coupled to ATP synthesis. This process is a significant source of energy in aerobic cells, and therefore inhibitors of the electron transport chain can be detrimental to the cell's metabolic processes.
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Electron Transport Chain: Complex I and II01:46

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The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
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Genetic polymorphisms in drug targets have emerged as critical determinants of interindividual variability in drug response and toxicity. Pharmacogenomic investigations increasingly focus on identifying these variations to personalize and optimize therapeutic interventions. A drug target may be a receptor, enzyme, or signaling protein involved in pharmacologic responses or disease-related pathways. While early pharmacogenetic studies focused primarily on drug metabolism, current research...

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Fingerprinting Cardiolipin in Leukocytes by Mass Spectrometry for a Rapid Diagnosis of Barth Syndrome
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L-carnitine in beta thalassemia.

Rashid Merchant1, Abhay Jain, Amish Udani

  • 1Department of Pediatrics, Dr Bhalabhai Nanavati Hospital, Mumbai, India. deandoc2000@hotmail.com

Indian Pediatrics
|May 12, 2009
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Summary

Beta thalassemia patients exhibit lower L-carnitine levels compared to healthy individuals. This deficiency may correlate with iron overload, highlighting the need for L-carnitine monitoring in these patients.

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Published on: November 4, 2018

Area of Science:

  • Biochemistry
  • Hematology
  • Nutritional Science

Background:

  • Beta-thalassemia is a genetic blood disorder requiring regular blood transfusions.
  • Transfusion therapy can lead to iron overload, a condition with significant health implications.
  • L-carnitine plays a crucial role in energy metabolism and is influenced by various physiological factors.

Purpose of the Study:

  • To investigate and compare L-carnitine levels in beta-thalassemia patients undergoing regular transfusions and chelation therapy versus healthy controls.
  • To explore the potential correlation between L-carnitine levels and iron status (ferritin levels) in beta-thalassemia patients.

Main Methods:

  • A comparative study involving 40 beta-thalassemia patients (mean age 17.5 years) and 10 age-matched healthy controls.
  • Quantitative measurement of serum L-carnitine levels using a defined assay.
  • Assessment of serum ferritin levels to evaluate iron status in patients.

Main Results:

  • Beta-thalassemia patients demonstrated significantly lower mean L-carnitine levels (23.71 microM) compared to controls (29.26 microM; P<0.0001).
  • Patients with ferritin levels exceeding 2000 ng/dL showed significantly lower L-carnitine levels (22.80 microM) than those with ferritin below 2000 ng/dL (30.1 microM; P=0.037).
  • Dietary habits (vegetarian vs. non-vegetarian) did not show a statistically significant impact on L-carnitine levels in this cohort (P=0.072).

Conclusions:

  • Regularly transfused and chelated beta-thalassemia patients have demonstrably lower L-carnitine levels than age-matched healthy individuals.
  • Elevated iron stores, indicated by high ferritin levels, are associated with reduced L-carnitine concentrations in beta-thalassemia.
  • These findings suggest a potential link between iron overload and L-carnitine deficiency in beta-thalassemia, warranting further investigation and consideration for supplementation.