Fixing A Fatal Genetic Defect In Babies With A Bit Of Genetic Modification

Genetic defects are exceedingly common, which is not surprising considering just how many cells make up our bodies, including our reproductive cells. While most of these defects have no or only minor effects, some range from serious to fatal. One of these defects is in the CPS1 gene, with those affected facing a shortened lifespan along with intensive treatments and a liver transplant as the only real solution. This may now be changing, after the first successful genetic treatment of an infant with CPS1 deficiency.

Carbamoyl phosphate synthetase I (CPS1) is an enzyme that is crucial for breaking down the ammonia that is formed when proteins are broken down. If the body doesn’t produce enough of this enzyme in the liver, ammonia will accumulate in the blood, eventually reaching levels where it will affect primarily the nervous system. As an autosomal recessive metabolic disorder it requires both parents to be carriers, with the severity depending on the exact mutation.

In the case of the affected infant, KJ Muldoon, the CPS1 deficiency was severe with only a low-protein diet and ammonia-lowering (nitrogen scavenging) medication keeping the child alive while a search for a donor liver had begun. It is in this context that in a few months time a CRISPR-Cas9 therapy was developed that so far appears to fixing the faulty genes in the liver cells.

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Mouse Model Suggests Starch-Based Plastics Are Still Bad For You

To paraphrase The Simpsons: plastics are the solution to – and cause of – all of mankind’s problems. Nowhere is this more clear in the phenomenon of microplastics. Some have suggested that alternative bioplastics made out of starch could be the solution here, as the body might be able to digest and disassemble these plastic fragments better. Unfortunately, a team of Chinese researchers put this to the test using mice, with the results suggesting that starch-based plastics do not change the harm to tissues and organs.

We previously looked at this harm from micro- and nanoplastics (MNP), with humans and their brains at autopsy showing a strong correlation between disease and presence of MNPs. In this recent study mice were split up into three groups, for either no, low or high levels of these bioplastics in their food. At autopsy, the mice exposed to the bioplastics all showed damage to organs, including the same gene-regulation issues and inflammation markers as seen with other plastics.

Despite these results, researchers question how useful these results are, as they pertain to modified starches with known biodegradability issues, while starch by itself is absolutely digestible when it’s in the form of potato chips, for instance. Perhaps the trick here is to make bioplastics that are still useful as plastics, and yet as harmless to ingest as said potato chips.

Not that we recommend eating bioplastics, mind you; potato chips are definitely tastier.

Two telescopes looking into the night sky.

Making A Backyard Observatory Complete With Retractable Roof

Here’s one for our astronomy geeks. Our hacker [arrow] has made their own observatory!

This particular video is a bit over ten minutes long and is basically a montage; there is no narration or explanation given, but you can watch clear progress being made and the ultimate success of the backyard facility.

Obviously the coolest thing about this building is that the roof can be moved, but those telescope mounts look pretty sexy too. About halfway through the video the concrete slab that was supporting one metal mounting pole gets torn up so that two replacements can be installed, thereby doubling the capacity of the observatory from one telescope to two.

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The French Chinon nuclear power plant with its low-profile, forced-draft cooling towers. (Credit: EDF/Marc Mourceau)

Recovering Water From Cooling Tower Plumes With Plume Abatement

Electrostatic droplet capture system installed on an HVAC condenser. (Credit: Infinite Cooling)

As a common feature with thermal power plants, cooling towers enable major water savings compared to straight through cooling methods. Even so, the big clouds of water vapor above them are a clear indication of how much cooling water is still effectively lost, with water vapor also having a negative impact on the environment. Using so-called plume abatement the amount of water vapor making it into the environment can be reduced, with recently a trial taking place at a French nuclear power plant.

This trial featured electrostatic droplet capture by US-based Infinite Cooling, which markets it as able to be retrofitted to existing cooling towers and similar systems, including the condensers of office HVAC systems. The basic principle as the name suggests involves capturing the droplets that form as the heated, saturated air leaves the cooling tower, in this case with an electrostatic charge. The captured droplets are then led to a reservoir from which it can be reused in the cooling system. This reduces both the visible plume and the amount of cooling water used.

In a 2021 review article by [Shuo Li] and [M.R. Flynn] in Environmental Fluid Mechanics the different approaches to plume abatement are looked at. Traditional plume abatement designs use parallel streams of air, with the goal being to have condensation commence as early as possible rather than after having been exhausted into the surrounding air. Some methods used a mesh cover to provide a surface to condense on, while a commercially available technology are condensing modules which use counterflow in an air-to-air heat exchanger.

Other commercial solutions include low-profile, forced-draft hybrid cooling towers, yet it seems that electrostatic droplet capture is a rather new addition here. With even purely passive systems already seeing ~10% recapturing of lost cooling water, these active methods may just be the ticket to significantly reduce cooling water needs without being forced to look at (expensive) dry cooling methods.

Top image: The French Chinon nuclear power plant with its low-profile, forced-draft cooling towers. (Credit: EDF/Marc Mourceau)

Gene Editing Spiders To Produce Red Fluorescent Silk

Regular vs gene-edited spider silk with a fluorescent gene added. (Credit: Santiago-Rivera et al. 2025, Angewandte Chemie)
Regular vs gene-edited spider silk with a fluorescent gene added. (Credit: Santiago-Rivera et al. 2025, Angewandte Chemie)

Continuing the scientific theme of adding fluorescent proteins to everything that moves, this time spiders found themselves at the pointy end of the CRISPR-Cas9 injection needle. In a study by researchers at the University of Bayreuth, common house spiders (Parasteatoda tepidariorum) had a gene inserted for a red fluorescent protein in addition to having an existing gene for eye development disabled. This was the first time that spiders have been subjected to this kind of gene-editing study, mostly due to how fiddly they are to handle as well as their genome duplication characteristics.

In the research paper in Angewandte Chemie the methods and results are detailed, with the knock-out approach of the sine oculis (C1) gene being tried first as a proof of concept. The CRISPR solution was injected into the ovaries of female spiders, whose offspring then carried the mutation. With clear deficiencies in eye development observable in this offspring, the researchers moved on to adding the red fluorescent protein gene with another CRISPR solution, which targets the major ampullate gland where the silk is produced.

Ultimately, this research serves to demonstrate that it is possible to not only study spiders in more depth these days using tools like CRISPR-Cas9, but also that it is possible to customize and study spider silk production.

Plugging Plasma Leaks In Magnetic Confinement With New Guiding Center Model

Although the idea of containing a plasma within a magnetic field seems straightforward at first, plasmas are highly dynamic systems that will happily escape magnetic confinement if given half a chance. This poses a major problem in nuclear fusion reactors and similar, where escaping particles like alpha (helium) particles from the magnetic containment will erode the reactor wall, among other issues. For stellarators in particular the plasma dynamics are calculated as precisely as possible so that the magnetic field works with rather than against the plasma motion, with so far pretty good results.

Now researchers at the University of Texas reckon that they can improve on these plasma system calculations with a new, more precise and efficient method. Their suggested non-perturbative guiding center model is published in (paywalled) Physical Review Letters, with a preprint available on Arxiv.

The current perturbative guiding center model admittedly works well enough that even the article authors admit to e.g. Wendelstein 7-X being within a few % of being perfectly optimized. While we wouldn’t dare to take a poke at what exactly this ‘data-driven symmetry theory’ approach exactly does differently, it suggests the use machine-learning based on simulation data, which then presumably does a better job at describing the movement of alpha particles through the magnetic field than traditional simulations.

Top image: Interior of the Wendelstein 7-X stellarator during maintenance.

The Make-roscope

Normal people binge-scroll social media. Hackaday writers tend to pore through online tech news and shopping sites incessantly. The problem with the shopping sites is that you wind up buying things, and then you have even more projects you don’t have time to do. That’s how I found the MAKE-roscope, an accessory aimed at kids that turns a cell phone into a microscope. While it was clearly trying to appeal to kids, I’ve had some kids’ microscopes that were actually useful, and for $20, I decided to see what it was about. If nothing else, the name made it appealing.

My goal was to see if it would be worth having for the kinds of things we do. Turns out, I should have read more closely. It isn’t really going to help you with your next PCB or to read that tiny print on an SMD part. But it is interesting, and — depending on your interests — you might enjoy having one. The material claims the scope can magnify from 125x to 400x.

What Is It?

The whole thing is in an unassuming Altoids-like tin. Inside the box are mostly accessories you may or may not need, like a lens cloth, a keychain, plastic pipettes, and the like. There are only three really interesting things: A strip of silicone with a glass ball in it, and a slide container with five glass slides, three of which have something already on them. There’s also a spare glass ball (the lens).

What I didn’t find in my box were cover slips, any way to prepare specimens, and — perhaps most importantly — clear instructions. There are some tiny instructions on the back of the tin and on the lens cloth paper. There is also a QR code, but to really get going, I had to watch a video (embedded below).

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