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(Image caption: This image of a neuron in tissue culture reveals an extensive network of filaments. Credit: GerryShaw/Wikimedia Commons)

The Molecular Biology Behind ALS

By now, most everyone has seen videos all over social media of friends and family dousing themselves in ice cold water as part of the ALS Ice Bucket Challenge.

The 2014 campaign was part of an effort to raise awareness of amyotrophic lateral sclerosis, or ALS, a devastating neurodegenerative disorder that causes progressive muscle weakening and loss of coordination. But what made much less of a splash in the media is what researchers are doing to tackle the issue.

ALS is notoriously difficult to treat, and relatively little is known about exactly how and why it occurs. In a rare discovery, a clear molecular defect has been found at the junctions between neurons and muscles, which may provide greater insight into the fundamental mechanisms of ALS, according to a new study by Daniela Zarnescu, associate professor in the University of Arizona’s Department of Molecular and Cellular Biology, and Alyssa Coyne, a graduate student in the UA’s Neuroscience Graduate Interdisciplinary Program and first author on the study. The paper is published in the Journal of Neuroscience.

In healthy people, nerve cells called motor neurons make contact with muscle fibers at places called neuromuscular junctions, which allows for appropriate control of movement and other critical functions. In ALS patients, motor neurons die off in droves, preventing these connections from occurring.

To study ALS, Zarnescu and Coyne use the fruit fly Drosophila melanogaster as their model, which gives the researchers the advantage of molecular and genetic approaches that allow them to more easily pinpoint exactly when and where things go wrong.

"When you tell people that you use the fruit fly as a model of human disease, you get some funny looks," Zarnescu said. "But using simplistic models can help you uncover what’s really important in the context of the disease."

Zarnescu and Coyne studied a protein called TDP-43, which previously has been implicated in ALS. The team found that TDP-43 regulates the creation and transport of another protein called Futsch at the neuromuscular junction. In the ALS model, TDP-43 prevents Futsch from making it to the neuromuscular junction, which results in a faulty connection.

"Alyssa discovered that this particular molecule is not regulated properly. It’s not made in the right place or in the right amount," Zarnescu said. "Instead of being transported to the neuromuscular junction, it stays in the body of the cell and can’t maintain the stability of the connection."

The researchers then wanted to determine if increasing the amount of Futsch protein would help repair the poor connection. Astoundingly, overexpressing Futsch in motor neurons had the effect of increasing the stability of the connection, increasing the lifespan of motor neurons and restoring motor function in the ALS fruit flies.

At this point, you might be asking: What does ALS in the nervous system of the fly have to do with ALS in humans?

To find out, Zarnescu and Coyne collaborated with researchers at the Barrow Neurological Institute in Phoenix to look at cells from the spinal cords of human ALS patients. The team looked at a protein called MAP1B, which is the mammalian version of the Futsch protein. Remarkably, the localization of MAP1B was altered in a very similar manner to the Futsch protein in fruit flies. The similarities suggest comparable defects in both human and fly models of ALS.

"This highlights the importance of studying human disease in simple models," Zarnescu said. "These models are extremely powerful, and predictive of defects that occur in human patients."

According to Zarnescu and Coyne, the findings represent a major step forward in understanding and eventually treating the disease.

"This study is among the first to highlight such a clear molecular defect at synaptic connections in ALS," Zarnescu said. "We don’t yet know exactly what is going on in ALS, but this discovery provides a possible explanation."

youtube

"Video/documental que muestra de manera cruda y realista, reacciones y opiniones sobre la desaparición de los 43 estudiantes de Ayotzinapa."

"Video/documentary that shows in a crude and real way reactions and opinions about the disappearance of the 43 Ayotzinapa students"

[gets warning tht my comps gonna shut off in 15 mins] Boy, do I love Ibuki Mioda. Sometimes, when I’m home alone, I’ll take some Ibuki Mioda fresh out of the oven and rub them in my scalp. It doesn’t do much for my hair health, but I like the way they feel running through my strands of hair. The scene phase, smooth black hair, and warmth.

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Mexico’s Day of Rage

43 college students were abducted on Sept. 26th in Guerrero, Mexico. Police and gunmen shot 6 students to death before taking the other 43. Mexico has taken some action by arresting the “mayor of the city of Iguala, José Luis Abarca; his wife, María de los Ángeles Pineda; and an aide and charged them with masterminding the attack.”

Why aren’t more people talking about this?

(Pictures are from the link above)

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A peaceful rally over the presumed massacre of 43 missing students ended with violence and destruction in Mexico City, with protesters calling for the president to stand down.
Thousands marched along Mexico City’s main boulevard, chanting for Enrique Pena Nieto to resign and waving blackened flags of the country in anger over the case of the missing students. They echoed ‘you are not alone’ to parents of the missing who joined the rally at the Angel of Independence Monument.
Dec2 2014
Reuters/AFP/AP/BBC

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In México, we are all fighting against a president who can’t even keep us safe. 43 students from a rural university, between the ages of 18-30, were abducted and then murdered when they were on their way to a pacific event. Bullets came from municipal cops, and the order of shooting came directly from the governor of that particular region.
Federal authorities have been covering their asses and the families of the students have no information about their people. Not even the dead bodies have been retrieved to them.

That’s why several universities from all the country have taken action. 

We support the families and their claim for justice and peace. 

Their anger is ours.

We’re getting closer to justice for the 43 missing Mexican students 

The former mayor of the Mexican city of Iguala has been charged with the kidnapping of 43 students feared to have been murdered last year, a step toward justice in the highly publicized case that sparked violent demonstrations across the country last year.

Tomas Zeron, director of criminal investigations at the federal Attorney General’s office, told Reuters that prosecutors had obtained an arrest warrant for former Mayor José Luis Abarca and 44 others on charges of kidnapping the students.

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PSA

I’ve been seeing that my people are doing this when their blogs are usually about fandoms and art. I feel the need to do this as well.

I’ve been reblogging things about the situation in my country, if you have seen it you know it’s about the usual oppression, murdered innocent people, violence, crimes, protests. But not just that, me, my mother, my siblings, my friends, MEXICO is finally getting up against the government (Because everything that happens here is because of the fucked up system). I DON’T reblog those posts to SEEM as someone cult, I DO IT BECAUSE I CARE, IT’S NOT SOMETHING TO BRAG ABOUT. It’s worrying, it’s exhausting, it’s hurting but it’s something that AT LEAST EVERY MEXICAN wherever you are SHOULD care about. It hurts me that I know a few mexican followers that just prefer to ignore it because it’s not something that goes with their blog. Let me tell you something, it’s NOT going to fade away, it’s NOT something just of a few days, it’s NOT going to make everything alright, it’s NOT going to disappear just by being ignorant about it. But it COULD fade away, it COULD make everything alright and it COULD solve if you reblog and inform others, because after all it’s SUPPORT. JUST. SUPPORT. We have come to the point where finally the government fears our people, finally we are pressing them! Finally we’re achieving to kick out all the corrupt politicians! DON’T LEAVE US ALONE. WE COULD CHANGE. WE COULD SAVE LIVES if you just don’t ignore us.

You can blacklist it, because I also and of course, don’t think nor want to force you all to give a reblog, a like, anything. So thanks if you read this and thanks, a lot, if you help us. Today for us, tomorrow for you.