Validation of Nanosensor Oxygen Measurement

Inclusion Criteria:

• Inclusion Criteria for Healthy Controls

1. Males and females, between the ages of 18 and 65 years, inclusive

2. Provide informed consent for study participation; able to understand andcomplete the protocol

3. Able to ambulate independently

4. Able to perform bicycle ergometry

Inclusion Criteria for Mitochondrial Myopathy (MM) Cases

1. Males and females, between the ages of 18 and 65 years, inclusive

2. Provide informed consent for study participation; able to understand and completethe protocol

3. Genetically-confirmed MM as defined by a diagnosis of primary mitochondrial disease (PMD) with predominant symptoms of myopathy as expressed by exercise intolerance andmuscle weakness and fatigue.

4. Previously enrolled (or will enroll) in Children's Hospital of Philadelphia (CHOP)Institutional Review Board (IRB) study #08-006177 (Falk, PI) or CHOP IRB #16-013364 (Zolkipli, PI)

5. Able to ambulate independently

6. Able to perform bicycle ergometry

Exclusion Criteria:

Subjects will be excluded if any of the following apply:

1. Unable to provide informed consent and complete all study procedures, includingergometry

2. Non-ambulatory or unable to ambulate independently

3. Pregnant

4. Within 1 month of a recent hospital admission due to acute illness

5. Have severe cardiac disease as defined by an ejection fraction of less than 35% andNew York Heart Association Functional Classification Class III; or severe pulmonarydisease as defined by the need for supplemental O2 therapy or daytime ventilatorysupport

6. Have a tracheostomy

7. Have a known bleeding disorder and/or family history (first-degree relative) with aknown bleeding disorder

8. Daily intake of aspirin or any other anti-platelet therapy which cannot betemporarily discontinued for medical reasons

9. a) Have known or suspected congenital or acquired immune deficiency; b) concurrentuse of immunosuppressive drugs, including corticosteroids; c) past history ofrecurrent (more than 6 times per year) severe (required hospitalization) skin orsoft tissue infections; d) history of infection or delayed wound healing aftersurgery or biopsy; e) known history of neutropenia with absolute neutrophil countless than 500/mm3

10. Undergo chronic steroid treatment as defined by daily oral intake (for more than 1month) or have existing untreated endocrinopathies, such as hypothyroidism thatcaused acquired myopathy

11. Prone to hypertrophic scars and keloids

12. Have any other known inherited myopathy, such as Duchenne muscular dystrophy orcongenital myopathy

13. Known allergy to lidocaine

14. Have a cognitive impairment that may prevent the ability to complete studyprocedures

15. Unable to comply with the requirements of the study protocol and/or unsuitable forthe study for any reason, in the opinion of the principal investigator

16. Individuals from vulnerable populations (e.g., prisoners/detainees)

17. Participants who are unable to speak and/or read English (as participants will berequired to be proficient to complete study procedures)

18. Employed by the U.S. Department of Defense, including U.S. military personnel