Scientists Develop Therapeutic Protein, Protect Nerve Cells from Huntington’s Disease

A new scientific study reveals one way to stop proteins from triggering an energy failure inside nerve cells during Huntington’s disease. Huntington’s disease is an inherited genetic disorder caused by mutations in the gene that encodes huntingtin protein. Approximately 30,000 Americans have mutant huntingtin protein which can impair energy-producing parts of nerve cells called mitochondria. The mutant protein destroys nerve cells and slowly chips away at a person’s ability to walk, speak, and control their behavior. Xin Qi, PhD, assistant professor of physiology and biophysics at Case Western Reserve University School of Medicine has been looking for proteins that interact with mutant huntingtin to better understand the initial steps of Huntington’s disease progression.

“Because mitochondrial dysfunction has been proposed to play an important role in the pathogenesis of Huntington’s disease,” said Qi, “we investigated the binding proteins of mutant huntingtin on mitochondria.” His recent study published in Nature Communications characterized one protein, valosin-containing protein (VCP) that Qi’s research team found in high abundance inside nerve cell mitochondria. Qi and colleagues discovered that VCP is recruited to nerve cell mitochondria by mutant huntingtin protein.

The researchers showed that mice with mutant huntingtin had mitochondria full of VCP, as did nerve cells donated by people with Huntington’s disease. The VCP inside mitochondria only interacted with mutant, but not healthy huntingtin protein. According to Qi, “In Huntington’s disease, the VCP-mutant huntingtin binding is greatly increased. This abnormal binding causes more VCP accumulation on the mitochondria,” Nerve cells with VCP-mutant huntingtin interacting inside them became dysfunctional and self-destructed.

“We found that VCP is a key player in mitochondria-associated autophagy, a mitochondria self-eating process. Over-accumulation of VCP on mitochondria thus results in a great loss of mitochondria, which leads to neuronal cell death due to lack of energy supply.” explained Qi. The researchers worked to identify ways to prevent VCP from heading to nerve cell mitochondria and interacting with mutant huntingtin protein once inside.
The team identified the regions of VCP and mutant huntingtin that were interacting. They cleverly designed a small protein, or peptide, with the same regions to disrupt the VCP-mutant huntingtin protein interaction. In nerve cells exposed to their peptide, VCP and mutant huntingtin bound the peptide instead of each other. Nerve cells exposed to the novel peptide had healthier mitochondria than unexposed cells. In fact, the peptide prevented VCP from relocating to mitochondria at all, and prevented nerve cell death.

Qi wanted to determine if the peptide had more than subcellular effects, and if it could be used therapeutically to prevent Huntington’s disease symptoms. The researchers administered the peptide to mice with Huntington’s-like disease and assessed mouse motor skills. Huntington’s-like mice exhibit spontaneous movement including excessive clasping, poor coordination, and decreased lifespan. Mice treated with the novel peptide did not experience these symptoms and appeared healthy. Qi concluded that the peptide reduced nerve cell impairment caused by Huntington’s disease in the animal model.

The study successfully countered harmful effects of mutant huntingtin and protected nerve cells in several models of Huntington’s disease. According to Qi, the interfering peptide developed in the study “suggests a potential therapeutic option for treatment of Huntington’s disease, a disease with no treatment available.” The next step for the researchers will be to optimize the potentially therapeutic peptide for use in human studies.

najwa4517  asked:

Hello Can i req some render from my photo aikatsu photo on stage?

if you look at the blogs description as well as the ask-page, you’ll see requests are still closed at the moment. they will mostlikely be closed til the end of the month.
this is so i can finish the WM summer event renders, new trade-shop SR renders i’m behind in, and the VCP fullbody renders, as well as the beach-event fullbody PR renders from last year i didn’t get to and this year’s beach fullbody PRs.

Kabul, Afghanistan. 11 May 2005 - A Coyote armoured reconnaissance vehicle with the Kabul Multinational Brigade (KMNB) armoured reconnaissance squadron, drives past the ruins of the King’s Palace en route to a Vehicle Check Point (VCP) outside of Kabul, Afghanistan.

gonna finish up the VCP fullbody renders between tonight and tomarrow, then get on the WM beach-event SR part 1 renders afterwards.
will probably try to get somemore card PR renders out between this event and the drop event and possibly some summer-fullbody PR renders (like the two new ones, last years i havnt gotten to yet, and the two aistar ones out)

Daily Thoughts - VCP and Me

The good news is that I took the exam to become a VMware Certified Professional on vSphere 5 and I totally passed.  456 out of 500, 300 being a passing score.  That sounds pretty good, and I am glad b/c my job will pay for the exam as long as I pass.  I already had my certification on vSphere 4, so the exam did not hold much mystery for me in terms of preparation and format.  But, although I passed the exam I don’t feel like it measured my actual skill with vSphere in any meaningful way.

I work with VMware everyday, and while my knowledge may not be encyclopedic; it is deep in its breadth and scope.  In theory, I should be able to waltz into the exam room (1,2,3,4…1,2,3,4) and pass with flying colors.  This is not the actual case.  Instead I spent the last two weeks going over exam prep questions collected from,, and  This is cheating, straight up.  No moral obfuscation here.  Someone else has purloined the question pool for the exam and posted them online for me to review and memorize.  I feel conflicted about this.  Overall I try to be an upstanding moral person, I’m even trying to pay for music these days.  The idea of memorizing actual questions from the exam in order to pass it debases the value of the exam and by extension the certificate.  But is it necessary?

The questions that VMware puts on its exam are by turns esoteric and easily answered by a Google search.  There is no logical reason that I need to know how much space logging needs to handle 100 VMs and 10 hosts.  VMware provides a calculator to tell you on their site.  That should not be a test question.  In the same vein is a question regarding the space for the /var/log partition, the maximum number of VMs a host can have, and the max number of virtual disks per host.  This is important information, which is why it is well documented on VMware’s site, free of charge.  Honestly, if you are an IT professional and still haven’t figured out how to use “the Google box”, then it’s time to look at another field.

What is the point of a certification and what does it signal to future employers and your supervisor?  I thought the point of the certification was to show that you have a deep understanding of the core concepts and operation of a technology.  You are able to effectively administer the system and troubleshoot problems.  Having a certification should say to prospective employers, “Yes I know this technology well, and will be able to perform its related functions at a high level.”  To your supervisor it says, “I have taken the time to thoroughly learn this topic and you can trust me to execute operations correctly and effectively.  I am a resource in this field for you and others.”

What does the certificate actually say?  "I spent two weeks memorizing questions and various arcane errata that will be only marginally relevant in a production environment.“ 

That sucks.  But it’s the reality, and may people do not realize it.  It sort of feels like VMware, Cisco, Microsoft, et al. know it and they have made their peace with it.  Although the test prep websites I mentioned are either very cheap or free, there is a separate test prep industry that makes millions off certification preparation.  And for those in upper management and HR that aren’t in the know, they continue to place a high value on having certificates and may even make hiring decisions based on their presence or absence.

It would be awesome if a cetificate exam actually proved the things I mentioned above.  And it could.  There are exams that involve a lab environment, where you have to prove your knowledge by accomplishing a set of goals.  This is expensive and harder to grade than a multiple choice exam, and Microsoft and Cisco have rolled this out to limited success.  Since VMware is all about virtualization, shouldn’t they be able to provide me with a virtual lab?  One would think so, unless they have a bunch of VCPs running their exam department.

I am both bothered by this on an intellectual level, and at the same time I am aware from a pragmatic standpoint that certifications are necessary in my field.  So while I grumble and grouse, in the end I still get in line.

Now you must excuse me.  I have to start "studying” for the MCITP exams.