In iPSC-derived cardiomyocytes from children with DCMA, incubation with SS‑31 for 24 hours reversed the fragmented, abnormally shaped mitochondria.
In plain wordsIn lab-grown heart cells with the DCMA mutation, SS‑31 reversed broken-up, misshapen mitochondria.
- Peptide
- Elamipretide (SS-31)
- Outcome
- DecreaseMitochondrial fragmentation and abnormal shape
- Study
humann = 2
iPSC-derived cardiomyocytes from children with DCMA- Outcome group
- Mitochondrial function · Cardiovascular
- Studied dose
- Dose not stated · 24 hoursAs given in this study (human).
Source sentences
These abnormalities were reversible by incubation with SS-31 for 24 hours.
3 context sentences(design, population, supporting numbers)
Patient iPSCs carry the causative DNAJC19 mutation (rs137854888) found in the Hutterite population, and the iPSC-CMs demonstrated highly fragmented and abnormally shaped mitochondria associated with an imbalanced isoform ratio of the mitochondrial protein OPA1, an important regulator of mitochondrial fusion.
Peripheral blood mononuclear cells from 2 children with DCMA and severe cardiac dysfunction were reprogrammed into induced pluripotent stem cells (iPSCs).
Patient and control iPSCs were differentiated into beating cardiomyocytes (iPSC-CMs) using a metabolic selection strategy.