New genetic culprit found for early progressive hair loss

People with hypotrichosis simplex have an ordinary measure of hair after birth, however they begin losing it in early adolescence. This misfortune keeps on advancing with age.

In spite of the fact that specialists realize this is an inherited condition, up until this point, they have just recognized few of the potential hereditary guilty parties.

One of these is a change in the APCDD1 gene, which meddles with a flagging pathway that impacts cell capacity and recovery. 

Presently, scientists from the University Hospital of Bonn in Germany, in a joint effort with associates from different organizations in Germany and Switzerland, have surrounded another quality that assumes a critical job in this uncommon kind of male pattern baldness.

Their investigation, the discoveries of which show up in The American Journal of Human Genetics, demonstrate that transformations in the LSS quality drive the components that portray hypotrichosis simplex.

Read the full article with the links 1 and 2

Restoring Hearing & Balance

Normal development of an ear(left) and abnormal ear due to loss of hair cells(right)

Our inner ears all contain hair cells which are important for hearing and balance. The final number of hair cells we have in our inner ear are reached before we are even born. Over time loud noises, trauma, infections, and aging cause us to lose these hair cells which impair our hearing and balance. Around 90% of hearing loss occurs when hair cells or auditory nerves are destroyed. Around 36 million adults in the U.S. have reported some degree of hearing loss. A new study from Rockefeller University has found that two genes responsible for inner ear development can be switched on to generate more hair cells in the ear and reverse the loss of hearing and balance. The study look place at the sensory neuroscience lab at Rockefeller University, headed by post-doctoral researcher Dr. Ksenia Gnedeva. Dr. Gnedeva began her study by looking for changes in gene expression in the utricle (an inner ear structure) of mice before and after birth. She discovered that two genes were highly active before birth but later became silent after birth. The silencing of these two genes after birth is what causes hair cells to stop developing. When both of these genes were switched off in the mice, the entire ear developed abnormally. However, when Dr. Gnedeva switched on the two genes in elder mice, she found that new hair cells began to generate inside the utricles of the inner ear. Dr.Gnedeva says that she hopes her findings will allow us to generate new hair cells later on in life in order to repair the loss of hearing and balance as we age.

I was surprised to learn that 36 million people in the U.S. suffer from some sort of hearing loss. It was interesting to learn that switching on two genes in the inner ear have the possibility to restore hearing and balance later on in life. It will be interesting to see if the same results seen in the mice will also be seen in humans.

Original Article

Inner ear gene discoveries offer clues about hearing and balance

Normal development on the left, absence of Sox11 and Sox4 leading to malformation of the inner ear structures on the right.

A new study from the Rockefeller University in New York may show researchers ways to regrow hair cells and restore lost hearing and balance. The study was lead by senior author and professor, A. James Hudspeth, and the lab work was done by Dr. Ksenia Gnedeva. They examined mice before and after birth, and discovered that two genes control the process of generating hair cells. By switching the genes on, new hair cells were observed in mature utricles. The utricle, a small sac or bag-like organ lined with hair cells that detects motion, is highly controlled by the transcription Sox4 and Sox11. Even in older mice, when the genes are turned on, Gnedeva found new hair cells developing. However, if both genes are turned off in developing mice, it leads to the entire inner ear developing abnormally.
This study was done in hopes to find a way to "restore hair cells later in life".
A study in a similar field was done recently, and it is the development of a genetic blueprint of inner ear cell development. This was found with a new technology called single-cell RNA-seq. This sequencing provides a' foundation for the potential development of cell-based therapies for treating hearing loss and balance disorders. The adaptation from the original media release can be found here.
I find this so interesting that such a small factor can have such an expansive outcome.

Discovery of Inner Ear Gene Grants Clues about Restoring Hearing and Balance

The inner ear contains hair cells that are important for hearing and balance, however, the number of hair cells an individual has is reached before we are born. Thereafter, loud noise, trauma, infections and aging will take their toll until loss of hair cells impairs hearing and balance. However, a new mouse study from The Rockefeller University in New York, NY could help researchers look at new ways to regrow hair hair cells and and restore lost hearing and balance. The study took place at Rockefeller and was done by a postdoctoral researcher, Dr. Ksenia Gnedeva. She examined mice before and after birth and found two genes that could switch on the process of generating hair cells.

Dr. Gnedeva began her work by looking for changes in gene expression in the utricle, an inner ear structure. She spotted two genes that are highly active before birth, but become silent after birth, which coincides with a halt in the development of hair cells in the mice's utricles. The two genes code for the transcription factors Sox4 and Sox11, which help shape the destiny of precursor cells into their final cell types by regulating the activity of other genes. Dr. Gnedeva found when the two genes were switched off, the entire ear, not just the utricle, developed abnormally. When she turned on the genes in older mice, she found it led to the regeneration of hair cells inside fully developed utricles. In the picture above, the image on the right happened with the absence of Sox4 and Sox11, which led to severe malformation of the inner ear structures that contain hair cells. 

Hearing occurs when sound waves reach the structures inside your ear, where the sound wave vibrations are converted into nerve signals that your brain recognizes as sound. Aging and exposure to loud noise may cause wear and tear on the hair cells in the inner ear. According to the CDC, Centers for Disease Control, around 36 million American adults report some degree of hearing loss. 36 million is a huge number and it is unfathomable to think about. Everyone should be able to hear because life without hearing can be severe. Imagine waking up everyday and not being able to hear. I cannot imagine myself not being able to hear. I hope Dr. Gnedeva does more research with these genes and maybe someday hearing loss will be a thing of the past.

Original article here