Primary Q10 deficiency is a rare dysfunction where the body’s missing ability to synthesize Q10 can lead to a rare type of nephrotic disease that cannot be treated with medicine. According to a new study that is published in Kidney International, however, it appears that Q10 supplementation can reduce the kidneys’ protein excretion, protect renal function, and lower mortality. Based on their findings, the scientists conclude that all patients with primary Q10 deficiency should ideally receive early and life-long Q10 therapy in order to prevent the disease from progressing and to avoid future organ damage.
Q10 is a coenzyme that is needed by the cellular mitochondria to produce energy. Q10 is also a powerful antioxidant that protects cells against oxidative stress. The more energy a cell generates, the more Q10 is needs. This is particularly true for cells in energy-demanding organs such as the heart and kidneys. We humans synthesize most of the Q10 that we need, but our endogenous synthesis decreases with age.
Primary Q10 deficiency is a very rare dysfunction caused by defects in the genes that are involved in the synthesis of Q10. This can lead to treatment-resistant nephrotic syndrome caused by failing kidney function.
In their new study, which is published in Kidney International, the scientists gathered and analyzed information from 116 patients with nephrotic syndrome, all of whom were all given supplements of Q10 for treating their primary Q10 deficiency. More specifically, the patients had defects in the following genes: COQ2, COQ6, or COQ8B. The average follow-up period was two years. The scientists measured the effect on the patients’ proteinuria (protein excretion) and they also analyzed kidney survival in 41 patients younger than 18 years of age who had chronic nephrotic disease in stages 1-4. This was done at baseline and at the end of the study. Afterwards, the researchers compared the results with the placebo group that was matched with regard to genotype, age, kidney function, and proteinuria.
It turned out that Q10 supplementation significantly reduced proteinuria by 88 percent over the course of 12 months. Complete absence of proteinuria was primarily observed in patients with a flawed COQ6 gene. Q10 supplementation resulted in a significantly better protection of the kidney function. Five-year survival was 62 percent in the Q10 group and 19 percent in the placebo group. The participants in the Q10 group also had a better improvement of their general condition and neurological manifestations.
The researchers concluded that all patients with primary Q10 deficiency should receive early and life-long Q10 therapy in order to slow the progression of kidney disease and to prevent further damage to other organs.
For the record, it pays off to choose a pharmaceutical-grade Q10 product because it is quite difficult for the body to absorb Q10, in the first place. So far, the only formulation with reliable documentation for its absorption and bioavailability is one with Q10 dispersed in a special type of oil that has been exposed to a heat treatment. This process ensures that the Q10 molecules are properly absorbed through the intestinal membrane.
What effect does Q10 have on nephrotic disease?
Lack of Q10 can impair the energy turnover, result in oxidative damage, and cause the attacked cells to perform apoptosis (programmed cell death). A special type of kidney cells called podocytes (cells in the Bowman's capsule in the kidneys that wrap around capillaries of the glomerulus) are particularly vulnerable due to their high energy turnover. Therefore, dysfunctions in the energy-producing mitochondria of the podocytes can destroy the kidneys’ ability to filter the blood.
Nephrotic syndrome is characterized by excessive proteinuria and peripheral oedemas. Other symptoms include foamy urine, swelling around the eyes, fatigue, nausea, and loss of appetite. Left untreated, the disease can become life-threatening. Nephrotic syndrome is normally treated with diuretics, ACE-inhibitors, and glucocorticoids. As mentioned earlier, however, patients with primary Q10 deficiency are treatment-resistant.
Q10 therapy has been shown to have promising potential. Although there has been some uncertainty concerning the long-term effect, this new study suggests that everyone with primary Q10 deficiency and nephrotic syndrome be treated with Q10 as soon as possible to optimize the body’s need for the compound and to prevent the disease from progressing. The authors also mention that Q10 support the mitochondria and other organ functions.
Stefania Drovandi et al. Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiency. Kidney International. September 2022
Illenia Cirilli et al. Role of Coenzyme Q10 in Health and Disease: an Update on the last 10 years (2010-2020). Antioxidants 2021
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