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Study · Animal study (in vivo) · mouse · 2022

3 results

Beneficial effects of SS-31 peptide on cardiac mitochondrial dysfunction in tafazzin knockdown mice.

Russo S, De Rasmo D, Signorile A, Corcelli A, Lobasso S

Scientific reports18 Nov 2022Cited by 26 (Europe PMC)

PMID36400945 (opens in a new tab)PMCIDPMC9674582 (opens in a new tab)DOI10.1038/s41598-022-24231-4 (opens in a new tab)PepBankPUB-36400945
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Summary

Barth Syndrome (BTHS), a genetic disease associated with early-onset cardioskeletal myopathy, is caused by loss-of-function mutations of the TAFAZZIN gene, which is responsible for remodeling the mitochondrial phospholipid cardiolipin (CL). Deregulation of CL biosynthesis and maturation in BTHS mitochondria result in a dramatically increased monolysocardiolipin (MLCL)/CL ratio associated with bioenergetic dysfunction. One of the most promising therapeutic approaches for BTHS includes the mitochondria-targeted tetrapeptide SS-31, which interacts with CL. Here, we used TAFAZZIN knockdown (TazKD) mice to investigate for the first time whether in vivo administration of SS-31 could affect phospholipid profiles and mitochondrial dysfunction. The CL fingerprinting of TazKD cardiac mitochondria obtained by MALDI-TOF/MS revealed the typical lipid changes associated with BTHS. TazKD mitochondria showed lower respiratory rates in state 3 and 4 together with a decreased in maximal respiratory rates. Treatment of TazKD mice with SS-31 improved mitochondrial respiratory capacity and promoted supercomplex organization, without affecting the MLCL/CL ratio. We hypothesize that SS-31 exerts its effect by influencing the function of the respiratory chain rather than affecting CL directly. In conclusion, our results indicate that SS-31 have beneficial effects on improving cardiac mitochondrial dysfunction in a BTHS animal model, suggesting the peptide as future pharmacologic agent for therapy.
CC BYText from Europe PMC. Highlighted sentences are the source of results.

Results from this study3

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

  1. 01AnimalAnimal study (in vivo)Efficacy outcome

    In TAFAZZIN knockdown mice, SS‑31 improved cardiac mitochondrial respiratory capacity.

    In plain wordsIn mice with a Barth syndrome-like defect, SS‑31 improved how well heart mitochondria use oxygen.

    Peptide
    Elamipretide (SS-31)
    Outcome
    IncreaseMitochondrial respiratory capacity
    Study

    mousen: not reported in source

    TAFAZZIN knockdown (TazKD) mice, a Barth syndrome model
    Outcome group
    Mitochondrial function · Cardiovascular
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Treatment of TazKD mice with SS-31 improved mitochondrial respiratory capacity and promoted supercomplex organization, without affecting the MLCL/CL ratio.
    1 context sentence
    Summary · context
    Here, we used TAFAZZIN knockdown (TazKD) mice to investigate for the first time whether in vivo administration of SS-31 could affect phospholipid profiles and mitochondrial dysfunction.
  2. 02AnimalAnimal study (in vivo)Mechanism

    In TAFAZZIN knockdown mice, SS‑31 promoted respiratory supercomplex organization in cardiac mitochondria.

    In plain wordsIn mice with a Barth syndrome-like defect, SS‑31 improved the organization of respiratory complexes in heart mitochondria.

    Peptide
    Elamipretide (SS-31)
    Outcome
    IncreaseRespiratory supercomplex organization
    Study

    mousen: not reported in source

    TAFAZZIN knockdown (TazKD) mice, a Barth syndrome model
    Outcome group
    Mitochondrial function · Cardiovascular
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Treatment of TazKD mice with SS-31 improved mitochondrial respiratory capacity and promoted supercomplex organization, without affecting the MLCL/CL ratio.
    1 context sentence
    Summary · context
    Here, we used TAFAZZIN knockdown (TazKD) mice to investigate for the first time whether in vivo administration of SS-31 could affect phospholipid profiles and mitochondrial dysfunction.
  3. 03AnimalAnimal study (in vivo)Mechanism

    In TAFAZZIN knockdown mice, SS‑31 did not change the monolysocardiolipin/cardiolipin ratio.

    In plain wordsIn mice with a Barth syndrome-like defect, SS‑31 did not change the cardiolipin ratio in heart mitochondria.

    Peptide
    Elamipretide (SS-31)
    Outcome
    No significant changeMLCL/CL ratio
    Study

    mousen: not reported in source

    TAFAZZIN knockdown (TazKD) mice, a Barth syndrome model
    Outcome group
    Mitochondrial function · Cardiovascular
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Treatment of TazKD mice with SS-31 improved mitochondrial respiratory capacity and promoted supercomplex organization, without affecting the MLCL/CL ratio.
    1 context sentence
    Summary · context
    Here, we used TAFAZZIN knockdown (TazKD) mice to investigate for the first time whether in vivo administration of SS-31 could affect phospholipid profiles and mitochondrial dysfunction.

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

    9 Oct 2026, 18:17 UTC

    Study results updated

  2. Version 1

    9 Oct 2026, 02:47 UTC

    First added · source update from Europe PMC

Subjects

Terms as the source lists them.

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