Spatax Meeting - Papers, Day 1

I had wondered how to present my observations from the conference, and my decision was to present by topic, chronologically - so this post covers the papers presented on the first day. The papers were a mix of HSP and Ataxia papers, and I focused on paying most attention to the HSP papers.

Full post index:
Overview: http://hspjourney.blogspot.co.uk/2016/06/international-meeting-on-spastic.html
Papers Day 1: http://hspjourney.blogspot.co.uk/2016/07/spatax-meet-papers-day-1.html
Papers Day 2a: http://hspjourney.blogspot.co.uk/2016/07/spatax-meeting-papers-day-2-part-1.html
Papers Day 2b: http://hspjourney.blogspot.co.uk/2016/08/spatax-meeting-papers-day-2-part-2.html
Papers Day 3: http://hspjourney.blogspot.co.uk/2016/08/spatax-meeting-papers-day-3.html
Posters 1: http://hspjourney.blogspot.co.uk/2016/08/spatax-meeting-poster-sessions-part-1.html
Posters 2: http://hspjourney.blogspot.co.uk/2016/09/spatax-meeting-poster-sessions-part-2.html

The meeting was opened - Henry Wahlig (of the Tom Wahlig Foundation) indicated that the foundation is awarding a scholarship of €120,000 over 3 years for scientists who are opening up new therapies or treatments into HSP. Further info here: http://www.hsp-info.de/stiftung/foerderpreis/advanced_scholarship_2016.htm

The first speaker was Harry Orr http://www.pathology.umn.edu/bio/lab-med-and-pathology-faculty/harry-orr. Harry talked about SCA1 (Spinocerebellar ataxia type 1), noting that the longer the delay in treatment the less able cells are to recover function.  Harry also noted a course in molecular degeneration in 2017 near Cambridge https://registration.hinxton.wellcome.ac.uk/events/item.aspx?e=606

 Andrea Burgo http://www.univ-evry.fr/fr/recherche/les_laboratoires/laboratoire_structure_et_activite_des_biomolecules_normales_et_pathologiques/andrea_burgo.html talked about SPG4, noting that SPG4 accounts for up to 50% of the dominant cases of HSP and up to 15% of the sporadic cases. He observed that the stability and viability of neurons depends on intracellular transport, and that axonal swelling is often associated with axonal transport defects,

Harald Stenmark http://cancell.no/principal-investigators-pis/harald-stenmark/ indicated that SPG10 may no longer be considered an SPG.

Julien Branchu https://www.researchgate.net/profile/Julien_Branchu reported on research undertaken in SPG11. They are developing a mouse model of SPG11. This model is a "knockout" model, where the gene is made inactive. They observed that the mice developed gait problems similarly to patients, and they also had cognitive/brain and spinal cord impairments, which are also characteristic of SPG11. The model will allow them to understand more about the condition.

As I write this I realise that it is on a very different level to my normal posts. I know I've just used a lot of terms that I dont normally use, and I've just kept this post to the points which I perceived to be most important.

Key Takeaway points;

• The longer the delay in treatment the less effective it is
• Animal models can greatly help the understanding of diseases

One of the terms that cropped up a lot was Purkinje cells - thse are cells that in the cerebellum which are used in processes of motor control and learning. The cerebellum is the part of the brain which has plays an important role coordinating and regulating muscle activity http://www.healthline.com/human-body-maps/cerebellum. It is more difficult to find an accesible description of the Purkinje cells https://embryo.asu.edu/pages/purkinje-cells