Axonal degeneration refers to the deterioration or damage of The axon, which is a part of the nerve cell. It can result from various causes like injury or disease, and it disrupts the communication between nerve cells, impacting their function.
Full definition
«The idea that caspases are even involved
in axonal degeneration during development is very recent» said Dr. Philip Barker, a principal investigator at The Neuro and senior author of the study.
«We want to
understand axonal degeneration at the same level that we understand programmed cell death, in the hopes of developing drugs to block the process when it becomes overactive,» DiAntonio says.
«
Treating axonal degeneration could potentially help a lot of patients because there are so many diseases and conditions where axons are inappropriately lost,» says Aaron DiAntonio, MD, PhD, professor of developmental biology.
«We want to understand
axonal degeneration at the same level that we understand programmed cell death, in the hopes of developing drugs to block the process when it becomes overactive,» DiAntonio said.
While axonal degeneration appears to be a major culprit in diseases like multiple sclerosis, it also paradoxically plays an important role in properly wiring the nervous systems of developing embryos.
Experiments conducted in mice and in human ALS cells reveal that when RIPK1 is out of control, it can spark axonal damage by setting off a chemical chain reaction that culminates in stripping the protective myelin off of axons and
triggering axonal degeneration — the hallmark of ALS.
Severing axons, or axotomy, is a simple way to study the molecular basis of neurodegeneration as it leads to the activation of
explosive axonal degeneration.
Dr. Barker and his colleagues show that the activity of certain «executioner» caspases (caspase - 3 and caspase - 9)
induce axonal degeneration and that their action is suppressed by a protein termed XIAP (X-linked inhibitor of apoptosis).
In the current study, Freeman and colleagues identified Axed, showed that it functions downstream of SARM to
execute axonal degeneration, and, surprisingly, that the protection afforded by blocking Axed was even stronger than SARM.
Richard T. Libby, PhD University of Rochester Medical School, Rochester, NY Funded by The Alcon Foundation Project: Understanding
Axonal Degeneration Pathways in Glaucoma
«
Treating axonal degeneration could potentially help a lot of patients because there are so many diseases and conditions where axons are inappropriately lost,» said Aaron DiAntonio, MD, PhD, professor of developmental biology.
There was no evidence
of axonal degeneration, demyelination, or abnormalities of supporting structures in the peripheral nerve evaluations, excluding a congenital polyneuropathy.
«We found that caspase - 3 - and -9 play crucial roles
in axonal degeneration and that their activities are regulated by XIAP.
In recent years, it is increasingly recognized that mDA
axonal degeneration may be central to the progression of the early stages of PD [4,5].
Axonal degeneration of dopamine - producing neurons is central to the progression of the early stages of PD.
The proband underwent two nerve biopsies which showed
an axonal degeneration, myelin modifications, and intra-axonal mitochondria with distorted cristae.