Possible Breakthrough for Sufferers of Huntington’s
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I don’t often get the chance to take a long hard look at medical news, but with the new year has brought the chance to write more and more for CFP. So it was with great anticipation that I looked at the news that two Indiana University Bloomington scientists had made progress in the cause to cure Huntington’s disease.
Published in the February 1st issue of Journal of Molecular Biology, IU Bloomington biologists Joel Ybe and Qian Niu describe a region on the surface of HIP1 (Huntingtin-interacting protein 1) that could bind HIPPI (HIP1-protein interactor). The association of HIP1 and HIPPI is believed to lead to the degeneration of nerve cells.
“If we now think that this is the region where HIPPI binds, we could eventually design a drug that can come in and sit down between these two proteins, which would prevent the binding of HIPPI,” said Ybe.
The paper is the first ever to put under the microscope a Huntington’s disease-related protein’s structure and function at the molecular level. Ybe, who led the research, and his colleagues hope that further and meticulous study will eventually lead to a cure.
“The important thing for us is to come up with something that will potentially help people,” said Ybe. “What is happening before the manifestation of the disease? Can we use this information to come up with drugs to diffuse that process?”
Huntington’s is a rare inherited neurological disorder that, while there have been major strides forwards in our understanding of it, still remains incurable. Wikipedia notes that it affects approximately 1 person per 10,000 people of Western European descent and 1 per 1,000,000 of Asian and African descent.
A heredity disorder – which means that it is passed down through a parents genes to their children – Huntington’s causes a large sum of nerve cells to die. Symptons include uncontrolled movements, dementia and depression, but these symptoms do not show themselves until those with Huntington’s reach 30 or 40 years of age.
From a biological standpoint, Huntington’s is caused by the huntingtin (correct spelling) protein (Htt) detaching from HIP1. The subsequent vacancy created by this allows another protein, HIPPI, to then bind to HIP1. The researchers found though that instead of the HIP1 protein containing a domain with a similar position to that of a death effector domains (DED) – which is then linked to proteins involved with apoptosis, a form of programmed cell death – the binding surface was a “coiled coil”, a fold in proteins coiled together like a rope.
With this new information reshaping what many thought to be true of Huntington’s, researchers hope to be able to finally trace which protein connections are the culprits in setting off the chain reaction which leads to the massive cell loss that is Huntington’s.
The research pair used X-ray crystallography to look at a specific area of interest upon the surface of HIP1. The targeted location works in conjunction with clathrin to convey nutrients in to a cell, and has long been associated with the development of Huntington’s.
As for X-ray crystallography, according to my good friend Wikipedia, it is ‘the science of determining the arrangement of atoms within a crystal from the manner in which a beam of X-rays is scattered from the electrons within the crystal.’ That being said, I imagine that the crystal can be swapped with biological matter to achieve the same goal, determining the arrangement of atoms within any form.
“Until we understand the relationship between huntingtin protein, HIP1, clathrin and HIPPI—we are not going to understand what is happening in the person who has the disease,” says Ybe. “You understand what’s going on in healthy cells, before you understand what’s going on in diseased cells.”
Joshua Hill, a Geek’s-Geek from Melbourne, Australia, Josh is an aspiring author with dreams of publishing his epic fantasy, currently in the works, sometime in the next 5 years. A techie, nerd, sci-fi nut and bookworm.