Barging In

There are a set of instructions in your body which make a specific gate (the science name for this is “ion channel“). Around 200,000 Dinky Amigos work together to make the instructions for this gate.

Some of these Dinky Amigos are not always on their best behaviour. There’s barging and shoving, swapping and disappearing altogether! Sometimes this leads to a disease called Cystic Fibrosis.  Scientists currently know of 2028 ways in which badly behaved Dinky Amigos can cause this disease. We would like to tell you about some of them.

 

Pushing and Shoving

Tristan

 

 

Hi – Tristan here. Normally Alina gets to do all the talking, so I thought I’d get in first

We’re all here in the CFTR gene (which is what scientists call our group. We’re the ones that make instructions for this gate). Some of the most badly behaved Dinky Amigos cause a bit of trouble every now and then.

To make your instructions, we Dinky Amigos have to line up together a certain order.

Sequence

Some of the other Dinky Amigos that are not in our group get a bit jealous. So they BARGE IN to our line. They don’t ask permission, they just pile right in and stand where they want to. Scientists call this an “insertion“.

There are nearly always problems when this happens because it messes up our instructions. (It’s a bit like someone taking a thick black pen and writing some gobbledegook in between the letters of a word in your favourite book. If you read it, that part of the bfghtthhook doesn’t make sense anymore.)

There are loads of these insertions. Mostly just one or two of the more boisterous Dinky Amigos muscle their way in for a laugh. But sometimes there is a bigger rush.

One day I was busily chatting to a cousin in the line, when EIGHT Dinky Amigos smashed their way in between us. This is really rare, but it is still very RUDE! What is worse, is that it caused my person to get Cystic Fibrosis.

Clinical Variation

Scientists made up a name for this behaviour. they called it 295ins8.

The “ins8” bit lets other scientists and doctors know just how rude those 8 Dinky Amigos have been. The “295” lets them know exactly where it has happened.

The name doesn’t help cure the cystic fibrosis but it does let doctors and other scientists know what to look out for if it happens again.

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