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Related Concept Videos

Inertia Tensor01:24

Inertia Tensor

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The concept of the inertia tensor is employed to depict the mass distribution and rotational inertia of a solid or rigid object. This tensor is expressed through a three-by-three matrix. Each component within this matrix corresponds to varying moments of inertia about specific axes.
The diagonal components of the inertia tensor matrix represent the moments of inertia concerning the principal axes of the object. These primary axes are defined as the axes where the object experiences the least...
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Diffusion01:12

Diffusion

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Diffusion is the passive movement of substances down their concentration gradients—requiring no expenditure of cellular energy. Substances, such as molecules or ions, diffuse from an area of high concentration to an area of low concentration in the cytosol or across membranes. Eventually, the concentration will even out, with the substance moving randomly but causing no net change in concentration. Such a state is called dynamic equilibrium, which is essential for maintaining overall...
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Diffusion01:21

Diffusion

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Diffusion is a type of passive transport. In passive transport, a substance tends to move from an area of high concentration to an area of low concentration until the concentration is equal across the space. For example, take the diffusion of substances through the air. When someone opens a perfume bottle in a room filled with people, the perfume is at its highest concentration in the bottle and is at its lowest at the edges of the room. The perfume vapor will diffuse, or spread away, from the...
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Facilitated Diffusion01:16

Facilitated Diffusion

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The plasma membrane, a critical structure in cellular biology, houses an array of transporters, or carrier proteins, interspersed within its lipid bilayer. These proteins play a crucial role in solute transport through facilitated diffusion, a form of passive diffusion that uses transporters to move the molecules across the membrane.
In this process, substrates such as organic compounds and ions interact with a transporter on one side, triggering conformational changes in proteins that enable...
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Behavior of Gas Molecules: Molecular Diffusion, Mean Free Path, and Effusion03:48

Behavior of Gas Molecules: Molecular Diffusion, Mean Free Path, and Effusion

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Although gaseous molecules travel at tremendous speeds (hundreds of meters per second), they collide with other gaseous molecules and travel in many different directions before reaching the desired target. At room temperature, a gaseous molecule will experience billions of collisions per second. The mean free path is the average distance a molecule travels between collisions. The mean free path increases with decreasing pressure; in general, the mean free path for a gaseous molecule will be...
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Protein Diffusion in the Membrane01:24

Protein Diffusion in the Membrane

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Proteins show rotational as well as lateral diffusion across the membrane. The lateral diffusion of proteins was confirmed through the cell fusion experiment where mouse and human cells were fused, resulting in hybrid cells. When the human and mouse cells fused, the specific membrane proteins on human and mouse cells were marked with the red and green-fluorescent markers, respectively. Initially, the red and green fluorescence was located on the respective hemisphere of the cell. As time...
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Diffusion Tensor Magnetic Resonance Imaging in Chronic Spinal Cord Compression
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Diffusion tensor imaging in metachromatic leukodystrophy.

Diane F van Rappard1,2, Marsh Königs3,4, Marjan E Steenweg1,2

  • 1Department of Pediatric Neurology, Center for Childhood White Matter Disorders, VU University Medical Center, Amsterdam, The Netherlands.

Journal of Neurology
|February 1, 2018
PubMed
Summary
This summary is machine-generated.

Diffusion tensor imaging reveals altered brain microstructure in metachromatic leukodystrophy (MLD) patients. These changes, including myelin loss and sulfatide accumulation, differ based on disease stage and hematopoietic cell transplantation eligibility.

Keywords:
Axial diffusivityDiffusion weighted imagingRadial diffusivitySulfatide storageWhite matter disorder

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Area of Science:

  • Neuroimaging
  • Neurology
  • Biochemistry

Background:

  • Metachromatic leukodystrophy (MLD) is a rare genetic disorder affecting myelin.
  • Understanding MLD pathomechanisms is crucial for developing effective treatments.

Purpose of the Study:

  • To investigate pathomechanisms of MLD using diffusion tensor imaging (DTI).
  • To compare diffusion measures between MLD patients and controls at diagnosis and during follow-up.
  • To assess the impact of hematopoietic cell transplantation (HCT) eligibility on diffusion measures.

Main Methods:

  • DTI was performed on 4 late-infantile, 16 juvenile, and 8 adult MLD patients, and 47 controls.
  • Fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) were quantified using tract-based spatial statistics (TBSS).
  • Diffusion measures were analyzed in normal-appearing white matter (NAWM), corpus callosum, thalamus, and pyramidal tracts, comparing HCT-eligible, non-eligible patients, and controls.

Main Results:

  • MLD patients showed decreased FA and increased MD and RD in white matter compared to controls.
  • Axial diffusivity (AD) was decreased in NAWM and corpus callosum but increased in the thalamus.
  • Differences were most significant in patients not eligible for HCT, with stable diffusion in treated patients and progressive abnormalities in non-eligible patients over 3.9 years.

Conclusions:

  • DTI findings confirm microstructural brain changes in MLD, indicating myelin loss and sulfatide accumulation.
  • Altered AD suggests a balance between myelin/axonal loss and intracellular sulfatide storage.
  • Diffusion measures provide insights into MLD progression and treatment response, particularly concerning HCT eligibility.