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Printable Handouts
Navigable Slide Index
- Introduction
- Summary (1)
- Intracellular protein trafficking
- Intracellular vesicle traffic
- Clinical syndromes with abnormal trafficking
- Intracellular trafficking model
- George Palade (1912-2008)
- Nobel lecture of George Palade
- Christian De Duve (1917-2013)
- Stuart Kornfeld
- James Rothman
- Randy Schekman
- Intracellular trafficking machinery
- Cargo recruitment and vesicle biogenesis
- Disorders of vesicle biogenesis
- Defects in Rabs and Rab-associated proteins
- Rab cycle
- Rab associated disorders
- Cytoskeleton and associated proteins
- Dynamic microtubules
- Mitochondria trafficked along microtubules
- Disorders of cytoskeletal traffic
- Kinesin movement along microtubules
- Vesicular tethering and fusion
- SNAREs and membrane fusion
- Disorders of defective vesicle fusion
- ARC syndrome
- ARC syndrome - liver damage
- ARC syndrome - kidney damage
- ARC platelets – a-granule defect
- Osteopenia, fractures, skin laxity
- VPS33B and VIPAR
- Intracellular localisation of VIPAR/VPS33B
- Cell model of ARC
- Reduced expression of E-cadherin in kd cells
- Transcriptional downregulation effect on E-cadherin
- Zebrafish model of ARC syndrome
- E-cadherin in human and zf VIPAR deficiency
- Conclusions: ARC syndrome
- Summary (2)
Topics Covered
- Concepts and pathways in intracellular trafficking
- Disorders affecting specific trafficking steps
- Arthrogryposis-Renal dysfunction-Cholestasis (ARC) syndrome
- ARC syndrome as an example of a trafficking disorder
Links
Series:
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Talk Citation
Gissen, P. (2015, January 19). Traffic problems: inherited disease and intracellular trafficking defect [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved December 21, 2024, from https://doi.org/10.69645/NTFN9523.Export Citation (RIS)
Publication History
Financial Disclosures
- Dr. Paul Gissen has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
A selection of talks on Cell Biology
Transcript
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0:00
My name is Paul Gissen,
and I'm a Researcher at
the UCL Laboratory for Molecular Cell Biology and Institute of Child Health.
I'm also a Consultant in metabolic medicine in Great Ormond Street Hospital for Children.
Today, I'm going to talk to you about traffic problems,
inherited disease, and intracellular trafficking defect.
0:24
In this talk, we will discuss concepts and pathways in intracellular protein trafficking.
We will also mention different disorders
that are caused by specific trafficking abnormalities.
I will also focus on ARC syndrome as an example
of a trafficking disorder as this is the interest of my laboratory.
0:51
Intracellular protein trafficking is broadly divided into two steps,
and they are directly opposite to each other.
One of them is protein secretion in which polypeptides which are synthesized
in the ribosomes are then folded and oligomerized in the ER,
and then packaged into transport vesicles in
the endoplasmic reticulum and travel to the Golgi complex.
In the Golgi, it's the organ where
post-translational modification occurs and then the proteins are sorted into
various routes and then directed in
various vesicles towards the membrane or other organelles of the cell.
The endocytosis is where the proteins are
internalized from the plasma membrane and then they can be recycled
back into the membrane or maybe trafficked for degradation to
lysosomes or transcytosed from the basal lateral to the apical membrane of the cell.
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