Chip’s Army: Fighting Against CLN3 Batten Disease

02/13/2026

Big news!

Batten-1 is a new proprietary and exclusive pharmaceutical product whose active ingredient is miglustat. The mechanism of action of this active ingredient blocks the accumulation of glycosphingolipids and neuroinflammation to prevent brain cell death. —Beyond Batten Disease Foundation

https://bdfa-uk.org.uk/news/news-thx-pharma-formally-theranexus-announce-partnership-biocodexo

News from THX Pharma (formally Theranexus) announce partnership with Biocodex

Yesterday we received news from THX Pharma (formally Theranexus) who have announced a partnership with Biocodex. They are continuing to lead the clinical development of Batten-1 in CLN3 Batten disease and hope to start a Phase 3 clinical trial later this year.

A copy of the news release (in English) can be found on our website: https://bdfa-uk.org.uk/news/news-thx-pharma-formally-theranexus-announce-partnership-biocodex

We look forward to sharing further news as we receive it.

02/11/2026

WE NEED YOUR VOTES: https://ow.ly/h4Yr50YcxSm

BDSRA has been nominated for Project for Awesome 2026. P4A is an annual event that raises awareness of charities that are doing good work across the world. P4A raises money to redistribute to charities voted on by our community. You will need to create an account to cast votes, and you may only vote once per charity per account!

02/09/2026

Good luck and thank you for all the support!! 🏈

02/05/2026

I read a post today that jumped up off my phone and slapped me. The main point was, “if it’s God’s will, it’s God’s bill.” It went on to say, sometimes we try to do everything ourselves, thinking we have to pay the price alone, but God also reminds us that when He calls, He also provides. Step forward. Surrender. And watch Him move.

I am taking it out of context a little bit… it didn’t really mean price or bill in the literal, monetary sense. But it struck a chord with me because I read that post in the pick up line at the pharmacy. Another $7,000 gone, but another month of medicine for Chip because God moved in all of you. We will never be able to thank all of you enough for the support you’ve shown our family, but I hope you know how much it means to us.

As far as an update, we started over fighting a new insurance company this month due to some expected changes with my job. So for now, the prayer request remains a short battle with this new insurance company. As far as the medicine goes, Chip is swallowing the pills like a champ… he acts like it’s a cool party trick!

When he came home today, he was so happy the snow was melted and there was plenty of mud to play in. He was even doing half-donuts in the front yard, despite what his daddy had to say about it! So thank you. Thank you for helping us at least try to delay his vision loss as long as we can, because this is WHY.

02/04/2026

✴ New insights into CLN3 protein and lysosomal function ✴

CLN3 disease is the most prevalent subtype of NCL caused by mutations in the CLN3 gene which leads to non-functional protein synthesis and lysosomal dysfunction. However, the precise role of CLN3 protein in lysosomal function remains poorly understood.

This new study reveals a critical role for CLN3 in regulating lysosomal chloride transport and cellular health, serving - at least in part - as a gatekeeper that regulates the internal environment of the lysosome. When the CLN3 protein is missing or non-functional, chloride (an ‘ion’ or particle that that helps control fluid balance, acidity, and electrical signals within cells) builds up in the lysosome, reducing its ability to clear cellular waste and leading to the toxic buildup of lysosomal storage material.

Excitingly, this research also identified that the protein CLN7 performs a similar, parallel role to CLN3, potentially explaining the convergent NCL pathologies seen in CLN3 and CLN7 diseases. Additionally the researchers also demonstrated that a promising curcumin-based compound called C1 could enhance CLN3 activity, improve lysosomal function, and activate TFEB, a master regulator of cellular clearance pathways.

Understanding CLN3’s role in lysosomal function brings researchers a step closer in developing targeted therapies that can restore cellular health for those living with CLN3 Batten disease.

👏 Congratulations to the authors: Yayu Wang, Kai Li, Wei Chen, Chao Chen, Adeline J.H. Yong, Xiaofan Zhang, Marena Tynan-La Fontaine, Yuh Nung Jan & Lily Yeh Jan.

👀 Read more about this research here: https://www.cell.com/neuron/fulltext/S0896-6273(25)00886-4

01/31/2026

The big game is almost one week away and there’s only 19 squares left!! Grab em’ while you can!!

01/30/2026

If you’re local, you know this stack of hay bales! If you’re not, the sweet mama that lives here paints these as something different every so often, usually to go with the season or next holiday. Chip always looks forward to seeing what’s on there when we go by.

Imagine his suprise when Chase AND Chip showed up this last week! We got to sneak in a quick photo before the snow hit.

Thank you so much! ❤️

01/22/2026

We’re halfway there… only 50 squares left! Help us complete the whole board! Thank you so much! 🙏🏻

01/20/2026

Jacob and I have been through some difficult days in our relationship; many… Jacob Chambers needs your support for Support Chip's Fight Against Batten Disease

01/20/2026