

A Huntington's Breakthrough? - mhb
http://pipeline.corante.com/archives/2014/03/28/a_huntingtons_breakthrough.php

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fraserharris
Interesting comment from the original article. I would appreciate if anyone
could more simply explain what these 8 diseases have in common:

"9\. Kelvin Stott on March 28, 2014 11:57 AM writes...

I did my PhD on the molecular mechanism of HD with Nobel laureate Max Perutz
back in 1993-6. We were trying to testing his "polar zipper" hypothesis,
whereby expanded glutamine repeats aggregate into extended b-sheets that are
inherently toxic, much like other amyoid-related diseases. And in fact, the
mutant protein has been shown to form non-specific pores in cell membranes,
just like other amyloid proteins.

That was many years ago, but anyway, I'd still like to share my perspective on
this...

The problem I see with the mechanism proposed in this Nature paper is seems
too specific for HD and huntingtin, however at least SEVEN other
neurodegenerative diseases (SCA1, SCA2, SCA3/MJD, DRPLA, Kennedy's Disease,
SBMA, etc.) have been linked to the expansion of glutamine repeats in
completely unrelated proteins.

To me this is just too much of a coincidence for each disease to be explained
by a different mechanism, and I think any mechanism for HD must be shown to
occur in all these other diseases before I would be prepared to believe it.

Just my 2c."

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refurb
The author of that comment is just saying that several other neurodegenerative
diseases (the 7 he listed) are so similar to Huntington's disease in terms of
disease progression and genetic traits (the glutamine repeats) that it seems
unlikely that the glutamine repeats in Huntington's act through a specific
enzyme, where for the other seven diseases the toxicity is due to amyloid
formation (and the resulting impact via pore formation in membranes).

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killertypo
grandpa has HD, dad has HD, and likely I will as well. Maybe someday we won't
have to worry about this disease :(...

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voidlogic
>>If I knew someone with the Huntingtin mutation, I believe that I would tell
them to waste no time taking cysteine supplements

Based on this finding are you going to take cysteine supplements?

Do you know if cysteine supplements have side effects?

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barlescabbage
Rule of the news, is there a question mark in the headline title? The answer
is always no.

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kyberias
Does not apply. This is not a news article per se, but a blog post. :)

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asdkl234890
It's a known _reputable_ source. The fact that it is also a blog, is not truly
related to that.

