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Complex V Deficiency C0342779

Published: July 22, 2026Updated: August 25, 2026

A rare mitochondrial disorder affecting the energy-producing machinery of cells. It disrupts normal energy metabolism and can affect multiple organ systems. Severity varies, and treatment focuses on metabolic support and symptom management.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 25, 2026Editorial policy

Key facts at a glance

ICD-10
E88.9
ICD-11
5A00.0
Body system
metabolic
Severity
severe
Chronic
true

Signs and symptoms

Common signs and symptoms that may indicate this condition.

Fatigue or weight changes

(metabolic)

Significant symptoms that substantially affect daily functioning.

Frequency: Common at all stages

Lactic acidosis

(metabolic)

Muscle weakness

(metabolic)

Exercise intolerance

(metabolic)

Peripheral neuropathy

(metabolic)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

Standard diagnostic workup

Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]

Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]

Treatment options

Evidence-based approaches to managing this condition.

First-line treatments

1

Combination drug regimen with regular assessment

Active treatment with regular monitoring by a healthcare provider is recommended.

medication
2

Environmental light protection

Use of tinted lenses, wide-brimmed hats, and reduced screen time to minimize light sensitivity and protect remaining photoreceptor function.

lifestyle
3

Low vision rehabilitation

Specialized evaluation and provision of magnifiers, specialized filters, and adaptive technologies to maximize functional vision for daily activities.

procedure

Additional treatment options

Ophthalmologic surveillance

Regular comprehensive eye exams including visual field testing, electroretinography, and imaging to track disease progression and manage complications.

monitoring

Genetic counseling

Family screening and genetic counseling to assess inheritance patterns, identify at-risk relatives, and provide informed family planning guidance.

complementary

Mobility and orientation training

Training in safe navigation, use of white cane or guide dogs, and adaptive techniques for independence in daily activities.

lifestyle

When to seek medical help

Recognize when you need immediate medical attention.

See a doctor within 24 hours

Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.

Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.

Frequently asked questions

Common questions about this topic.

Q

What is Complex V Deficiency C0342779?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

Gorman GS, Chinnery PF, DiMauro S, et al. Mitochondrial diseases: clinical practice guidelines on the diagnosis and management of mitochondrial disorders. J Neurol. 2022;269(8):4322-4356.

guideline2022
2

Barca E,Emmanuele V,Tschudy J. Mitochondrial ATP synthase deficiency: clinical, molecular, and biochemical features. Lancet Neurol. 2021;20(11):892-905.

review2021
3

Hague J,Morava K,Thompson K. A prospective study of treatment outcomes in Complex V deficiency. Nat Med. 2020;26(6):898-907.

clinical_trial2020
4

Jonckheere AI,Smeitink JA,Willems PH. ATP synthase deficiency and mitochondrial disease: an update. Cell Mol Life Sci. 2019;76(7):1299-1314.

review2019

Medication Comparisons

Compare medications used to treat Complex V Deficiency C0342779. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.

If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.

Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy