Skip to content

Study · In vitro (cells) · 2017

9 results

Peptide SS-31 upregulates frataxin expression and improves the quality of mitochondria: implications in the treatment of Friedreich ataxia.

Zhao H, Li H, Hao S, Chen J, Wu J, Song C, Zhang M, Qiao T, Li K

Scientific reports29 Aug 2017Cited by 59 (Europe PMC)

PMID28852135 (opens in a new tab)PMCIDPMC5575096 (opens in a new tab)DOI10.1038/s41598-017-10320-2 (opens in a new tab)PepBankPUB-28852135
Download PDFPubMedAsk about this study
Jump to 9 results

Summary

Friedreich ataxia is a progressive neurodegenerative disease caused by the expansion of GAA trinucleotide repeats within the first intron of the FXN gene, which encodes frataxin. The pathophysiology of the disease is thought to be derived from the decrease of Fe-S cluster biogenesis due to frataxin deficiency. There is currently no effective treatment for the disease. In our study, we demonstrated that treatment with the mitochondrion-targeted peptide SS-31 reduced frataxin deficiency-induced oxidative stress in lymphoblasts and fibroblasts derived from patients. Interestingly, SS-31 treatment translationally upregulated the protein level of frataxin in a dose-dependent manner. Furthermore, SS-31 treatment increased the enzymatic activities of the iron-sulphur enzymes, including aconitase and complex II and III of the respiratory chain. Further evaluation of the quality of mitochondria showed that mitochondrial membrane potential, ATP content, NAD+/NADH, and the morphology of mitochondria all improved. Our results suggest that SS-31 might potentially be a new drug for the early treatment of Friedreich ataxia.
CC BYText from Europe PMC. Highlighted sentences are the source of results.

Results from this study9

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

  1. 01LabIn vitro (cells)Mechanism

    In patient-derived lymphoblasts and fibroblasts with frataxin deficiency, SS‑31 reduced oxidative stress.

    In plain wordsIn lab tests on cells linked to Friedreich ataxia, SS‑31 reduced oxidative stress caused by low frataxin.

    Peptide
    Elamipretide (SS-31)
    Outcome
    DecreaseFrataxin deficiency-induced oxidative stress
    Study

    humann: not reported in source

    lymphoblasts and fibroblasts derived from patients with Friedreich ataxia
    Outcome group
    Mitochondrial function · Nervous system and cognition
    Studied dose
    Dose not stated

    Source sentences

    Summary
    treatment with the mitochondrion-targeted peptide SS-31 reduced frataxin deficiency-induced oxidative stress in lymphoblasts and fibroblasts derived from patients.
  2. 02LabIn vitro (cells)Mechanism

    In patient-derived cells, SS‑31 increased the protein level of frataxin in a dose-dependent manner.

    In plain wordsIn lab tests on cells linked to Friedreich ataxia, SS‑31 raised the level of frataxin protein, and more SS‑31 raised it more.

    Peptide
    Elamipretide (SS-31)
    Outcome
    IncreaseFrataxin protein level
    Study

    humann: not reported in source

    lymphoblasts and fibroblasts derived from patients with Friedreich ataxia
    Outcome group
    Mitochondrial function · Nervous system and cognition
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Interestingly, SS-31 treatment translationally upregulated the protein level of frataxin in a dose-dependent manner.
  3. 03LabIn vitro (cells)Mechanism

    In patient-derived cells, SS‑31 increased the enzymatic activity of the iron-sulphur enzyme aconitase.

    In plain wordsIn lab tests on cells linked to Friedreich ataxia, SS‑31 raised the activity of aconitase, an iron-sulphur enzyme.

    Peptide
    Elamipretide (SS-31)
    Outcome
    IncreaseAconitase enzymatic activity
    Study

    humann: not reported in source

    lymphoblasts and fibroblasts derived from patients with Friedreich ataxia
    Outcome group
    Mitochondrial function · Nervous system and cognition
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Furthermore, SS-31 treatment increased the enzymatic activities of the iron-sulphur enzymes, including aconitase and complex II and III of the respiratory chain.
  4. 04LabIn vitro (cells)Mechanism

    In patient-derived cells, SS‑31 increased the enzymatic activity of respiratory chain complex II.

    In plain wordsIn lab tests on cells linked to Friedreich ataxia, SS‑31 raised the activity of complex II of the energy-making chain.

    Peptide
    Elamipretide (SS-31)
    Outcome
    IncreaseRespiratory chain complex II activity
    Study

    humann: not reported in source

    lymphoblasts and fibroblasts derived from patients with Friedreich ataxia
    Outcome group
    Mitochondrial function · Nervous system and cognition
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Furthermore, SS-31 treatment increased the enzymatic activities of the iron-sulphur enzymes, including aconitase and complex II and III of the respiratory chain.
  5. 05LabIn vitro (cells)Mechanism

    In patient-derived cells, SS‑31 increased the enzymatic activity of respiratory chain complex III.

    In plain wordsIn lab tests on cells linked to Friedreich ataxia, SS‑31 raised the activity of complex III of the energy-making chain.

    Peptide
    Elamipretide (SS-31)
    Outcome
    IncreaseRespiratory chain complex III activity
    Study

    humann: not reported in source

    lymphoblasts and fibroblasts derived from patients with Friedreich ataxia
    Outcome group
    Mitochondrial function · Nervous system and cognition
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Furthermore, SS-31 treatment increased the enzymatic activities of the iron-sulphur enzymes, including aconitase and complex II and III of the respiratory chain.
  6. 06LabIn vitro (cells)Mechanism

    In patient-derived cells, SS‑31 improved mitochondrial membrane potential.

    In plain wordsIn lab tests on cells linked to Friedreich ataxia, SS‑31 improved the electrical charge across the energy-producing parts (mitochondria).

    Peptide
    Elamipretide (SS-31)
    Outcome
    IncreaseMitochondrial membrane potential
    Study

    humann: not reported in source

    lymphoblasts and fibroblasts derived from patients with Friedreich ataxia
    Outcome group
    Mitochondrial function · Nervous system and cognition
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Further evaluation of the quality of mitochondria showed that mitochondrial membrane potential, ATP content, NAD+/NADH, and the morphology of mitochondria all improved.
  7. 07LabIn vitro (cells)Mechanism

    In patient-derived cells, SS‑31 improved ATP content.

    In plain wordsIn lab tests on cells linked to Friedreich ataxia, SS‑31 improved the cell energy (ATP) content.

    Peptide
    Elamipretide (SS-31)
    Outcome
    IncreaseMitochondrial ATP content
    Study

    humann: not reported in source

    lymphoblasts and fibroblasts derived from patients with Friedreich ataxia
    Outcome group
    Mitochondrial function · Nervous system and cognition
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Further evaluation of the quality of mitochondria showed that mitochondrial membrane potential, ATP content, NAD+/NADH, and the morphology of mitochondria all improved.
  8. 08LabIn vitro (cells)Mechanism

    In patient-derived cells, SS‑31 improved the NAD+/NADH measure.

    In plain wordsIn lab tests on cells linked to Friedreich ataxia, SS‑31 improved a measure of cell energy balance (NAD+/NADH).

    Peptide
    Elamipretide (SS-31)
    Outcome
    Direction not reportedNAD+/NADH ratio
    Study

    humann: not reported in source

    lymphoblasts and fibroblasts derived from patients with Friedreich ataxia
    Outcome group
    Mitochondrial function · Nervous system and cognition
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Further evaluation of the quality of mitochondria showed that mitochondrial membrane potential, ATP content, NAD+/NADH, and the morphology of mitochondria all improved.
  9. 09LabIn vitro (cells)Mechanism

    In patient-derived cells, SS‑31 improved the morphology of mitochondria.

    In plain wordsIn lab tests on cells linked to Friedreich ataxia, SS‑31 improved the shape of the energy-producing parts (mitochondria).

    Peptide
    Elamipretide (SS-31)
    Outcome
    Direction not reportedMitochondrial morphology
    Study

    humann: not reported in source

    lymphoblasts and fibroblasts derived from patients with Friedreich ataxia
    Outcome group
    Mitochondrial function · Nervous system and cognition
    Studied dose
    Dose not stated

    Source sentences

    Summary
    Further evaluation of the quality of mitochondria showed that mitochondrial membrane potential, ATP content, NAD+/NADH, and the morphology of mitochondria all improved.

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

    10 Oct 2026, 08:19 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.

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