Skip to content

Study · Animal study (in vivo) · mouse · 2020

6 results

Short-term NAD+ supplementation prevents hearing loss in mouse models of Cockayne syndrome.

Okur MN, Mao B, Kimura R, Haraczy S, Fitzgerald T, Edwards-Hollingsworth K, Tian J, Osmani W, Croteau DL, Kelley MW, Bohr VA

NPJ aging and mechanisms of disease7 Jan 2020Cited by 47 (Europe PMC)

Summary

Age-related hearing loss (ARHL) is one of the most common disorders affecting elderly individuals. There is an urgent need for effective preventive measures for ARHL because none are currently available. Cockayne syndrome (CS) is a premature aging disease that presents with progressive hearing loss at a young age, but is otherwise similar to ARHL. There are two human genetic complementation groups of CS, A and B. While the clinical phenotypes in patients are similar, the proteins have very diverse functions, and insight into their convergence is of great interest. Here, we use mouse models for CS (CSA -/- and CSB m/m ) that recapitulate the hearing loss in human CS patients. We previously showed that NAD+, a key metabolite with various essential functions, is reduced in CS and associated with multiple CS phenotypes. In this study, we report that NAD+ levels are reduced in the cochlea of CSB m/m mice and that short-term treatment (10 days) with the NAD+ precursor nicotinamide riboside (NR), prevents hearing loss, restores outer hair cell loss, and improves cochlear health in CSB m/m mice. Similar, but more modest effects were observed in CSA -/- mice. Remarkably, we observed a reduction in synaptic ribbon counts in the presynaptic zones of inner hair cells in both CSA -/- and CSB m/m mice, pointing to a converging mechanism for cochlear defects in CS. Ribbon synapses facilitate rapid and sustained synaptic transmission over long periods of time. Ribeye, a core protein of synaptic ribbons, possesses an NAD(H) binding pocket which regulates its activity. Intriguingly, NAD+ supplementation rescues reduced synaptic ribbon formation in both CSA -/- and CSB m/m mutant cochleae. These findings provide valuable insight into the mechanism of CS- and ARHL-associated hearing loss, and suggest a possible intervention.
CC BYText from Europe PMC. Highlighted sentences are the source of results.

Results from this study6

1 result from studies that gave the peptide. No result from this study comes from people.

  1. 01AnimalAnimal study (in vivo)Efficacy outcome

    In CSA and CSB mutant cochleae, NAD+ supplementation rescued reduced synaptic ribbon formation.

    In plain wordsIn mice with CSA or CSB mutations, NAD+ supplementation restored reduced synaptic ribbon formation.

    Outcome
    IncreaseSynaptic ribbon formation
    Study

    mousen: not reported in source

    Cockayne syndrome mouse models
    Outcome group
    Neuroprotection · Nervous system and cognition
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Intriguingly, NAD+ supplementation rescues reduced synaptic ribbon formation in both CSA -/- and CSB m/m mutant cochleae.

Natural levels measured1

These results measure the body's own peptide. Nobody was given the peptide. They are not evidence about taking it. They are left out of key results, evidence counts and charts.

  1. 02AnimalEndogenous levelAnimal study (in vivo)Other

    In CSB mutant mice, natural cochlear NAD+ levels were reduced.

    In plain wordsIn mice with CSB mutations, natural cochlear NAD+ levels were reduced.

    Outcome
    DecreaseCochlear NAD+ levels
    Study

    mousen: not reported in source

    Cockayne syndrome mouse models
    Outcome group
    Mitochondrial function · Nervous system and cognition

    Source sentences

    Summary
    In this study, we report that NAD+ levels are reduced in the cochlea of CSB m/m mice and

    This result measures the body's own NAD+ (nicotinamide adenine dinucleotide). Nobody was given the peptide. It is not evidence about taking it.

Studies that did not test the peptide alone4

In this study, NAD+ (nicotinamide adenine dinucleotide) was a lab tool or a carrier, part of a mix, or the subject of a lab method. These results are not evidence about taking NAD+ (nicotinamide adenine dinucleotide).

  1. 03AnimalNot a test of NAD+ (nicotinamide adenine dinucleotide) aloneAnimal study (in vivo)Efficacy outcome

    In CSB mutant mice, the NAD+ precursor NR prevented hearing loss.

    In plain wordsIn mice with CSB mutations, the NAD+ precursor NR prevented hearing loss.

    Outcome
    DecreaseHearing loss
    Study

    mousen: not reported in source

    Cockayne syndrome mouse models
    Outcome group
    Neuroprotection · Nervous system and cognition

    Source sentences

    Summary
    short-term treatment (10 days) with the NAD+ precursor nicotinamide riboside (NR), prevents hearing loss, restores outer hair cell loss, and improves cochlear health in CSB m/m mice.

    In this study, NAD+ (nicotinamide adenine dinucleotide) was a lab tool or a carrier, part of a mix, or the subject of a lab method. This result is not evidence about taking NAD+ (nicotinamide adenine dinucleotide).

  2. 04AnimalNot a test of NAD+ (nicotinamide adenine dinucleotide) aloneAnimal study (in vivo)Efficacy outcome

    In CSB mutant mice, the NAD+ precursor NR reduced outer hair cell loss.

    In plain wordsIn mice with CSB mutations, the NAD+ precursor NR reduced outer hair cell loss.

    Outcome
    DecreaseOuter hair cell loss
    Study

    mousen: not reported in source

    Cockayne syndrome mouse models
    Outcome group
    Neuroprotection · Nervous system and cognition

    Source sentences

    Summary
    short-term treatment (10 days) with the NAD+ precursor nicotinamide riboside (NR), prevents hearing loss, restores outer hair cell loss, and improves cochlear health in CSB m/m mice.

    In this study, NAD+ (nicotinamide adenine dinucleotide) was a lab tool or a carrier, part of a mix, or the subject of a lab method. This result is not evidence about taking NAD+ (nicotinamide adenine dinucleotide).

  3. 05AnimalNot a test of NAD+ (nicotinamide adenine dinucleotide) aloneAnimal study (in vivo)Efficacy outcome

    In CSB mutant mice, the NAD+ precursor NR improved cochlear health.

    In plain wordsIn mice with CSB mutations, the NAD+ precursor NR improved cochlear health.

    Outcome
    IncreaseCochlear health
    Study

    mousen: not reported in source

    Cockayne syndrome mouse models
    Outcome group
    Neuroprotection · Nervous system and cognition

    Source sentences

    Summary
    short-term treatment (10 days) with the NAD+ precursor nicotinamide riboside (NR), prevents hearing loss, restores outer hair cell loss, and improves cochlear health in CSB m/m mice.

    In this study, NAD+ (nicotinamide adenine dinucleotide) was a lab tool or a carrier, part of a mix, or the subject of a lab method. This result is not evidence about taking NAD+ (nicotinamide adenine dinucleotide).

  4. 06AnimalNot a test of NAD+ (nicotinamide adenine dinucleotide) aloneAnimal study (in vivo)Efficacy outcome

    In CSA mutant mice, the NAD+ precursor NR had similar but more modest effects than in CSB mutant mice.

    In plain wordsIn mice with CSA mutations, the NAD+ precursor NR had similar but smaller effects than in mice with CSB mutations.

    Outcome
    MixedEffects in CSA mutant mice
    Study

    mousen: not reported in source

    Cockayne syndrome mouse models
    Outcome group
    Neuroprotection · Nervous system and cognition

    Source sentences

    Summary
    Similar, but more modest effects were observed in CSA -/- mice.
    1 context sentence
    Summary · context
    short-term treatment (10 days) with the NAD+ precursor nicotinamide riboside (NR), prevents hearing loss, restores outer hair cell loss, and improves cochlear health in CSB m/m mice.

    In this study, NAD+ (nicotinamide adenine dinucleotide) was a lab tool or a carrier, part of a mix, or the subject of a lab method. This result is not evidence about taking NAD+ (nicotinamide adenine dinucleotide).

AI wrote and checked these results. AI can make mistakes, so check important results against the source.

Version history

PepBank keeps every version of the data on this study. Status changes from Retraction Watch, Europe PMC and Crossref are listed with their source.

  1. Version 2Current

    14 Oct 2026, 05:02 UTC

    Study results updated

  2. Version 1

    14 Oct 2026, 01:46 UTC

    First added · source update from Europe PMC

Subjects

Terms as the source lists them.

PepBank is a research reference. Talk to a doctor before you use any peptide.