“Leaky” Connective Tissue in Ehlers-Danlos?

Hello, friends. Apologies for the somewhat long hiatus I’ve taken from blogging. I began teaching a couple classes this semester and most of my free time has been filled with Powerpoints and Zoom meetings galore. I’ve also had to say a very painful good-bye to my two furbaby twins, Teddy and Jamie, who finally succumbed to their Lysosomal Storage Disease. So, I’ve not only been incredibly busy, but also in mourning for my sweet special needs boys who became a major focal point of my life for the last year.

Nevertheless, as my heart continues to heal and the semester’s work is winding down, I now find myself with the occasional moment to breathe. And so I’m back blogging– albeit this is a short one today.

To give a little backstory, I’ve been reviewing some pathophysiology and came across something that I had long forgotten: Namely, that mesenchyme (connective tissue) acts as a physical and functional barrier to local defensive reactions (e.g., inflammation), preventing them from spilling into adjacent tissues and ultimately becoming a systemic reaction.

Given the nature of mast cell disorders in Ehlers-Danlos syndromes/hypermobility spectrum disorders (EDS/HSD)– especially the way a small potentially localized response can flare out of control, even to the point of anaphylaxis– it does beg the question whether the connective tissue in EDS/HSD is essentially “leaky.”

For instance, previous research has found that mesenchymal cells have immunosuppressive qualities and may be a potential target for various allergic diseases, autoimmune conditions, and even severe acute respiratory failure in COVID infection [1, 2, 3]. In essence, these cells are made to be immunosuppressive boundaries that keep inflammatory reactions contained.

Interestingly, this type of preliminary experiment could be done in a petri dish in order to determine whether EDS/HSD connective tissue is failing to contain localized reactions compared non-EDS/HSD tissue. So, for any of you cell biologists out there, feel free to steal the idea! 😉

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