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Enzymes02:34

Enzymes

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Inside living organisms, enzymes act as catalysts for many biochemical reactions involved in cellular metabolism. The role of enzymes is to reduce the activation energies of biochemical reactions by forming complexes with its substrates. The lowering of activation energies favor an increase in the rates of biochemical reactions.
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Enzymes speed up reactions by lowering the activation energy of the reactants. The speed at which the enzyme turns reactants into products is called the rate of reaction. Several factors impact the rate of reaction, including the number of available reactants. Enzyme kinetics is the study of how an enzyme changes the rate of a reaction.
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Intrinsically Disordered Proteins02:18

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Intrinsically disordered proteins are a group of proteins that do not fold into specific three-dimensional structures. Their structural flexibility allows them to complement ordered proteins to perform functions that are inaccessible to rigid structures. They are more common in eukaryotes than prokaryotes and may either be exclusively intrinsically disordered or hybrid proteins, consisting of a mix of ordered and disordered regions. The absence of a rigid structure in these proteins can be...
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Red Algae01:23

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Red algae, also known as rhodophytes, are primarily found in marine environments, though some species inhabit freshwater and terrestrial ecosystems. These organisms exist in both unicellular and multicellular forms, with some multicellular varieties reaching macroscopic sizes.As phototrophic organisms, red algae contain chlorophyll a; however, their chloroplasts lack chlorophyll b. Instead, they possess phycobiliproteins, which serve as major light-harvesting pigments, similar to those found in...
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Enzyme-linked Receptors01:00

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Enzyme-linked receptors are proteins that act as both receptor and enzyme, activating multiple intracellular signals. This is a large group of receptors that include the receptor tyrosine kinase (RTK) family. Many growth factors and hormones bind to and activate the RTKs.
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Enzyme Inhibition01:30

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Inhibitors are molecules that reduce enzyme activity by binding to the enzyme. In a normally functioning cell, enzymes are regulated by a variety of inhibitors. Drugs and other toxins can also inhibit enzymes. Some inhibitors bind to the enzyme’s active site, while others inhibit enzymatic activity by binding to other sites on the protein structure.
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Measuring Deformability and Red Cell Heterogeneity in Blood by Ektacytometry
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Measuring Deformability and Red Cell Heterogeneity in Blood by Ektacytometry

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Red Blood Cell Enzyme Disorders.

Rachael F Grace1, Bertil Glader2

  • 1Department of Pediatric Hematology/Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, 450 Brookline Avenue, Dana 3-106, Boston, MA 02215, USA.

Pediatric Clinics of North America
|May 28, 2018
PubMed
Summary
This summary is machine-generated.

Red blood cell enzyme disorders, like glucose-6-phosphate dehydrogenase deficiency, cause hemolysis and variable symptoms. Early diagnosis is crucial for managing neonatal jaundice, anemia, and iron overload.

Keywords:
AnemiaCholecystectomyGlucose-6-phosphate dehydrogenase deficiencyOther red cell enzyme disordersPyruvate kinase deficiencySplenectomy

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Area of Science:

  • Biochemistry
  • Hematology
  • Genetics

Background:

  • Mature red blood cells depend on the glycolytic pathway for energy and the hexose monophosphate shunt for oxidative stress protection.
  • Red blood cell enzyme disorders, though common, present with significant clinical variability.
  • Glucose-6-phosphate dehydrogenase deficiency is the most prevalent red cell enzyme disorder globally.

Purpose of the Study:

  • To summarize the clinical presentations and diagnostic importance of common red blood cell enzyme disorders.
  • To highlight the metabolic pathways essential for red blood cell function and protection.

Main Methods:

  • Literature review of red blood cell metabolism and enzyme deficiencies.
  • Analysis of clinical manifestations associated with specific enzyme disorders.
  • Emphasis on diagnostic criteria and management strategies.

Main Results:

  • Glucose-6-phosphate dehydrogenase deficiency commonly presents with neonatal jaundice and hemolytic anemia post-oxidative stress.
  • Pyruvate kinase deficiency and other glycolytic disorders can lead to chronic hemolytic anemia, jaundice, gallstones, and iron overload.
  • Clinical variability is a hallmark of these inherited red blood cell disorders.

Conclusions:

  • Accurate diagnosis of red blood cell enzyme deficiencies is essential for effective patient care.
  • Understanding these disorders aids in predicting and managing complications like hemolysis and iron overload.
  • Timely diagnosis facilitates appropriate monitoring and treatment, improving patient outcomes.