Researchers fight off childhood neurodegenerative disease characteristics rooted in NAD+ deficiency, mitochondrial damage, and senescence
Ataxia telangiectasia (A-T) is a devastating, complex genetic disorder characterized by degeneration of the nervous system often during infancy or early childhood. Premature aging diseases like A-T are often linked to breakdown and leakage of the battery packs that generate energy for our cells (mitochondria) and senescence — an arrest in cell growth and replication that’s necessary to keep our organs from decaying. These phenomena happen in natural aging too, but don’t take off so early in life and at such a rapid rate. But whether cells freeze up and mitochondria crash hasn’t been explored regarding A-T.
In an article published in Aging Cell, Yang and colleagues from the National Institute on Aging demonstrate that the buildup of damaged mitochondria and senescent cells occurs in cells from A-T patient as well as cultured human cells and mice that model the disease. The research team based out of Bethesda, Maryland, find that boosting levels of nicotinamide adenine dinucleotide (NAD+) — a molecule at the core of many processes, including mitochondrial function and recycling — clears damaged mitochondria and prevents senescence in A-T models.
These findings link the neurological symptoms of A-T directly to senescence and the loss of healthy mitochondrial populations within cells.