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Printable Handouts
Navigable Slide Index
- Introduction
- Mitochondria in the human cell
- Ultrastructure of the mitochondrion
- Mammalian mitochondrial carriers & metabolism
- The mitochondrial carrier family
- Lego model
- Topology model of the ADP/ATP carrier
- Structure of the yeast ADP/ATP carrier Aac3p
- Structure of inhibited bovine ADP/ATP carrier
- Symmetry analysis
- Properties of amino acids
- Replacement scores for membrane proteins
- Scoring symmetry in the phosphate carrier
- Symmetry analysis of the phosphate carrier
- ADP binding to the mitochondrial ADP/ATP carrier
- Substrate binding sites in mitochondrial carriers
- Properties of the common substrate binding site
- Binding occurs in the middle of the membrane
- Matrix salt bridges of odd-numbered α-helices
- Cytoplasmic salt bridges of even-numbered helices
- Key functional features of mitochondrial carriers
- Cytoplasmic and matrix salt bridge networks
- Strict equimolar ADP/ATP exchange
- The transport cycle of glutamate uniport
Topics Covered
- Mitochondria in the human cell
- Role of mitochondrial carriers in human metabolism
- Sequence and structure of mitochondrial carriers
- The central substrate binding site
- The involvement of salt bridge networks in the transport mechanism
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Talk Citation
Kunji, E.R. (2018, March 29). Mitochondrial transporters and disease - function and mechanism [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved December 5, 2024, from https://doi.org/10.69645/UHNX6771.Export Citation (RIS)
Publication History
Financial Disclosures
- Dr. Edmund R.S. Kunji has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
Mitochondrial transporters and disease - function and mechanism
Published on March 29, 2018
61 min
Other Talks in the Series: Mitochondria in Health and Disease
Transcript
Please wait while the transcript is being prepared...
0:00
Welcome everybody. My name is Edmund Kunji.
I work for the Medical Research Council
Mitochondrial Biology Unit of the University of Cambridge,
and today I'm going to talk about
Mitochondrial Transporters and how they're involved in their disease.
In my first talk,
I'm going to set the scene explaining what
mitochondrial carriers are and what they do in human physiology,
and then I'll go on and explain
the basic mechanism how we believe that they are actually working.
In my second talk,
I will explain the structural mechanism in much more detail,
and how these transporters are regulated.
But first, to set the scene,
I will explain what mitochondria actually are.
0:45
Mitochondria are organelles in the human cell.
What you see here is a closeup inside a human cell,
which contains a large number of different organelles.
To the right-hand top corner,
what you find is the nucleus,
this is where the genetic information is stored.
Just below that it's the endoplasmic reticulum,
where proteins are synthesized with the information obtained from
the genetic material and where they are shipped to other parts of the cell.
In the left-hand upper corner,
you'll find the golgi apparatus,
this is the post office of the human cell where proteins are
transported to and sorted to go into
different directions in the cell. Then you find here
on the left-hand bottom,
you find a lysosome,
this is where proteins are degraded to be recycled for other purposes.
Then here in the front,
you find on the right-hand side,
right and bottom side, the close up of what we call the mitochondria.
Now the mitochondria are the powerhouses of the cell,
but this I will show you in a minute,
they do a lot more than that.
What you also find is that these mitochondria actually form large networks,
that are highly dynamic that are involved in fission,
the split up of these networks and fusion, diffusion of these networks,
and it's through all of these that they maintain
a high number of functional mitochondria in the cell.
Mitochondria are essential for many purposes as I will explain in a minute.
But first, let me tell you what the basic structure of a mitochondrion is.
So, this actually shows here,