CMT 2: Unexpected Mitofusion fuction leads to potential therapies

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(Article in Italian. This is a rough English translation)

Like a sms launch from a part all' other of the cells

http://www.superando.it/content/view/4010/122/

The sense of a recent regarding study is own in this Mitofusin 2,

protein of which a new function has been discovered, that of " point "

between mitocondria - it centers them energetic of the cells - and

the endoplasmatico reticulum, other important cellular structure

assigned to produce good part of proteins and to store important

cellular messages. The best understanding than such mechanisms

could open interesting perspectives for the future therapies of the

disease of Charcot-Marie-Tooth of type 2, rare hereditary neuropathy.

The young investigator Luca Scorrano calls Mitofusin 2 and was

already famous to the scientists for its involvement in a rare

hereditary neuropathy, the disease of Charcot-Marie-Tooth of type 2:

now a study published in the review " Natures " from Luca Scorrano,

investigator of the Institute Telethon Dulbecco that works near the

Institute Veneto di Molecular Medicina (VIMM) of Padova, reveals a

new and unexpected function, that could suggest interesting

therapeutic perspectives.

Before the publication of this job, in fact, that one of regulating

the form was believed that is that Mitofusin 2 had essentially an

only task, and the movement of the mitocondria, centers them to the

energy of the cells. They slide and its group has instead

demonstrated like this protein functions also from bridge between

mitocondria and the endoplasmic reticulum, other important structure

of the cells assigned to produce good part of proteins, the " fire-

bricks of the vita " and to store soccer, one of the more important

cellular message.

In our organism, soccer works like a kind of " sms " that it comes

launch from a part all' other of the cell in order to say to the

various structures what must make. Of fact, but, it is as if the

mitocondria they were " sordi " to it marks them: they have therefore

need that this " sms " or launch from much neighbor. The strait

contact between mitocondria and endoplasmic reticulum just serves to

favor the transfer of soccer to the Mitocondri same and however, when

Mitofusin 2 is altered - like in the event of the patients affcted

with Type 2 of the disease of Charcot-Marie-Tooth - is incapable of

holding the two organelli together: consequently, the mitocondria

they receive very badly marks them to destined they.

The importance of the job of Slides is twofold. From a part, in fact,

it demonstrates in definitive way that these organelli must be held

entirety in order to communicate from each other effectively. From

the other it opens interesting perspectives for all the scientists

who study the mechanism with in which the nervous cells degenerate in

form 2 of the disease of Charcot-Marie-Tooth.

Quest' the last pathology - that it hits approximately a person on

10.000 - is characterized by weakness and atrophy of muscles, that

they appear like " smagriti " , and from reduced sensibility. The

symptoms leave generally from the feet and they are diffused

progressively towards l' high to legs, the thighs and hands leave of.

Deformations of the skeleton are frequent also, in particular of the

feet, than cables are introduced.

Now, comprising the described mechanism better, it will be able to be

worked effectively more in order to characterize possible therapeutic

strategies.