3.1.5 Defects in myelination and related diseases

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Из курса от партнера Peking University

Advanced Neurobiology I

260 оценки

Peking University

Advanced Neurobiology I

260 оценки

Hello everyone! Welcome to advanced neurobiology! Neuroscience is a wonderful branch of science on how our brain perceives the external world, how our brain thinks, how our brain responds to the outside of the world, and how during disease or aging the neuronal connections deteriorate. We’re trying to understand the molecular, cellular nature and the circuitry arrangement of how nervous system works. Through this course, you'll have a comprehensive understanding of basic neuroanatomy, electral signal transduction, movement and several diseases in the nervous system. This advanced neurobiology course is composed of 2 parts (Advanced neurobiology I and Advanced neurobiology II, and the latter will be online later). They are related to each other on the content but separate on scoring and certification, so you can choose either or both. It’s recommended that you take them sequentially and it’s great if you’ve already acquired a basic understanding of biology. Thank you for joining us!

Из урока

Morphology of the neuronal cell & Cellular and Molecular Biology of the Neuron

Let's learn more about the basic unit of the nervous system: the neuron.

3.1.1 A overview of neurons5:47

3.1.2 Real life situation: Aβ in Alzheimer’s disease8:06

3.1.3 Organelles and cytoskeleton in neurons8:26

3.1.4 Research: induction of the tunneling nanotube7:05

3.1.5 Defects in myelination and related diseases3:23

3.1.6 Synthesis and trafficking of neuronal proteins I10:13

3.1.7 Synthesis and trafficking of neuronal proteins II8:08

Познакомьтесь с преподавателями

Yan Zhang
Professor
School of Life Sciences, Peking University
Yulong Li
Research Fellow
School of Life Sciences, Peking University

So in the CNS system, besides neurons,

we also have other cell types, astrocytes, microglias, oligodendrocytes.

Oligodendrocytes can make the myelination and

defect in myelin proteins disrupt conductions of nerve signals.

This is the oligodendrocytes, making myelination around the axon.

So, the picture here, the middle part is the axon.

And then the dark part around it is the myelin sheet,

made by oligodendrocyte cell.

So as one mentioned, the axon is in the middle, and

then oligodendrocyte, or Shwan cell, grow longer and

longer, and then make the wrap around the axon,

so, eventually, make myelin sheet.

If some myelin protein is deleted, then we'll have very severe phenotype.

One transgenic mice is called a shiverer.

So, the shiverer is the partial deletion of myelin basic protein.

The shiverer phenotype is the mice will shiver all the time.

So it's called a shiverer.

And it cannot work steadily in the surface.

So if we put back this myelin basic protein,

we can partially rescue the phenotype of the shiverer.

So the myelin sheet, this is wild type.

The dark part is the myelin sheet.

And then the middle picture is from the shiverer mice,

so you can see the myelin sheet are almost gone

in this mice, and about putting back MBP,

this protein, myelin basic protein,

we can partially rescue the myelin sheet,

but not as good as wild type.

So in human, we can also have diseases related to myelination.

In human, there's a disease called Charcot-Marie-Tooth disease,

so the Charcot-Marie-Tooth disease is the duplication

region happened in the myelin basic protein.

So, in this picture, the left part is the normal allele,

and then the right part is the duplication happened within the MBP protein.

So in the Charcot-Marie-Tooth, this isn't a normal wild type.

And so, in the Charcot-Marie-Tooth, the myelin sheet get thinner,

so the patient show the instability in the movement.

So the mutations in the MBP, or myelin basic protein,

can produce, can induce the disease in the myelin sheet.

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