Targeting Autophagy-NAD Pathway Protects Cells in Niemann-Pick Type C1 Disease: Preclinical Findings

SynopsisImpaired autophagy, which leads to the buildup of misfolded proteins and damaged organelles, has been linked to many human diseases. Loss of autophagy in metabolically active cells can caus...

Nicotinamide Riboside Reverses Metabolic Dysfunction in Werner's Syndrome: Preclinical Findings

SynopsisWerner syndrome (WS) features premature aging and metabolic dysfunction, yet mechanisms remain unclear. This study found that loss of the WRN protein accelerates early fat cell formation in...

Nicotinamide Riboside Restores NAD+ and Improves Heart and Brain Function in Leigh Syndrome: Preclinical Findings

SynopsisMice lacking the mitochondrial complex I subunit Ndufs4 develop Leigh syndrome (LS), marked by serious brain and heart problems. This study found these mice have slow, irregular heartbeats ...

Nicotinamide Riboside Restores Mitochondrial Function in Alpers’ Syndrome: Preclinical Findings

SynopsisAlpers' syndrome is a devastating early-onset neurodegenerative disorder caused by POLG mutations impairing mitochondrial DNA replication. Patient-derived iPSCs were used to create brain or...

Nicotinamide Riboside Restores Exercise Capacity and Mitochondrial Function in Barth Syndrome: Preclinical Findings

SynopsisBarth syndrome, caused by tafazzin mutation, leads to mitochondrial dysfunction and severe exercise intolerance. In a Drosophila model, NR supplementation restored exercise capacity and mit...

Nicotinamide Riboside Protects Against Chronic Kidney Disease by Boosting Metabolism: Preclinical Findings

SynopsisChronic kidney disease (CKD) impairs kidney metabolism, including fatty acid breakdown, and is linked to low NAD+ levels. This study tested nicotinamide riboside (NR), a precursor of NAD+, ...

Nicotinamide Riboside Improves Mitochondrial Health in Werner Syndrome: Preclinical Findings

SynopsisWerner syndrome (WS) is a rare accelerated aging disease caused by mutations in the WRN gene, leading to metabolic problems and premature cell aging. This study found that loss of WRN incre...

Nicotinamide Riboside Improves Mitochondrial Function and Exercise Ability in ANT1-Deficiency: Preclinical Findings

SynopsisMitochondrial disorders are often characterized by muscle weakness and fatigue. Mutations in the heart-muscle adenine nucleotide translocator isoform 1 (ANT1) (or ANT1-deficiency) of both h...

Nicotinamide Riboside Corrects Metabolic Imbalances and Mitochondrial Dysfunction in Citrin Deficiency: Preclinical Findings

SynopsisCitrin deficiency (CD) is a genetic metabolic disorder caused by loss of the mitochondrial transporter CITRIN, which plays a role in the urea cycle and malate–aspartate shuttle. Patients wi...

Nicotinamide Riboside Boosts NAD+ to Improve Telomere Stability: Preclinical Findings

SynopsisTelomere biology disorders (TBDs), such as dyskeratosis congenita (DC), are characterized by critically short telomeres and often lead to bone marrow failure. A key metabolic molecule, NAD+...

NAD+ Deficiency Drives Kidney Dysfunction in Cockayne Syndrome: Preclinical Findings

SynopsisCockayne Syndrome (CS) is a premature aging disorder caused by mutations in the CSA and CSB genes, leading to problems like early aging, nerve damage, and kidney dysfunction. Cells from CS ...

Mitochondrial Dysfunction Linked to NAD+ Decline and DNA Repair Dysfunction: Preclinical Findings

SynopsisMitochondrial problems are common in aging and neurodegenerative diseases. In xeroderma pigmentosum group A (XPA), a disease caused by faulty DNA repair that leads to severe neurodegenerati...

Genetic Background Influences Nicotinamide Riboside Response in Barth Syndrome: Preclinical Findings

SynopsisBarth Syndrome is a rare genetic disorder caused by mutations in the TAFAZZIN (TAZ) gene, which affects mitochondrial function by disrupting cardiolipin remodeling. While clinical trials ar...

Effects of Nicotinamide Riboside on Spinal and Bulbar Muscular Atrophy (SBMA): Preclinical Findings

SynopsisPrevious studies have shown that in spinal and bulbar muscular atrophy (SBMA), a mutant protein called polyQ-expanded AR becomes improperly acetylated, and activating SIRT1, an NAD-dependen...

Counteracting CD38-Induced NAD+ Depletion Reduces Multiple Organ Fibrosis: Preclinical Findings

SynopsisSystemic sclerosis (SSc) involves fibrosis in multiple organs, but its cause is not fully understood. NAD+ decline with age and disease, often due to increased CD38 activity (an NADase), ma...

Cockayne Syndrome Proteins Maintain Mitochondrial Health Through NAD+ Signaling: Preclinical Findings

SynopsisCockayne syndrome (CS) is a rare premature aging disorder involving DNA repair defects and mitochondrial dysfunction. Across human, mouse, and nematode models, mitochondrial dysfunction was...

Nicotinamide Riboside-Containing β-Hydroxybutyrate Supplementation (NHS101) in Children with Multiple Acyl-CoA Dehydrogenase or Pyruvate Dehydrogenase Deficiency: Case Report

Objective To evaluate the efficacy of a new preparation of dextro-β hydroxybutyrate salt mixture containing NR (NHS101) in patients with metabolic disorders affecting energy metabolism, specificall...

Nicotinamide Riboside Restores NAD+ Levels and Improves Metabolism in Diastolic Heart Failure: Preclinical Findings

Synopsis Heart failure with left ventricular diastolic dysfunction is a growing health problem worldwide. This study looked at levels of NAD+, a key metabolic cofactor, in the hearts of patients w...

Nicotinamide Riboside Protects Against Liver Fibrosis: Preclinical Findings

Synopsis Nicotinamide riboside (NR) boosts NAD+ levels and offers protective effects in various diseases, but its role in liver fibrosis is unclear. This study tested NR’s ability to protect again...

Nicotinamide Riboside Improves Alzheimer's Features and DNA Repair: Preclinical Findings

Synopsis Alzheimer’s disease (AD) involves problems with energy metabolism and DNA repair, but how these contribute to key AD features is unclear. A DNA repair-deficient AD mouse model (3xTgAD/Pol...