Mitochondrial membrane protein-associated neurodegeneration

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ORPHA:289560OMIM:614298G23.0
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2Specialists8Treatment centers

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Overview

Mitochondrial membrane protein-associated neurodegeneration (MPAN) is a rare inherited brain disorder that belongs to a group of conditions called neurodegeneration with brain iron accumulation (NBIA). In MPAN, abnormal amounts of iron build up in specific areas of the brain, particularly a region called the basal ganglia, which helps control movement. This iron buildup gradually damages brain cells over time. MPAN is caused by changes (mutations) in the C19orf12 gene, which provides instructions for making a protein found in the membranes of mitochondria — the energy-producing structures inside cells. When this protein does not work properly, iron accumulates in the brain and causes progressive neurological problems. The main symptoms include difficulty walking and movement problems (such as stiffness, involuntary muscle contractions called dystonia, and spasticity), speech difficulties, vision loss due to optic nerve damage, and a gradual decline in thinking and memory skills. Psychiatric symptoms like depression, anxiety, and behavioral changes are also common. The disease typically begins in childhood or young adulthood and worsens over time. Currently, there is no cure for MPAN, and treatment focuses on managing symptoms and maintaining quality of life. Physical therapy, medications for movement problems, and supportive care are the main approaches used.

Also known as:

Key symptoms:

Difficulty walking and balance problemsMuscle stiffness and spasticityInvoluntary muscle contractions (dystonia)Slurred or difficult speechVision loss from optic nerve damageDecline in thinking and memory skillsPsychiatric symptoms like depression or anxietyBehavioral changesDifficulty swallowingBladder problemsTremor or involuntary movementsWeakness in the legsSeizures (in some cases)Progressive loss of independence

Clinical phenotype terms (26)— hover any for plain English
Abnormal globus pallidus morphologyHP:0002453
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Mitochondrial membrane protein-associated neurodegeneration.

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No actively recruiting trials found for Mitochondrial membrane protein-associated neurodegeneration at this time.

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Specialists

2 foundView all specialists →
TD
Thomas Klopstock, Prof. Dr.
Specialist
PI on 3 active trials
FT
Figen TUNCAY
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.How quickly is this disease likely to progress in my case or my child's case?,What therapies and medications can help manage the movement and psychiatric symptoms?,Should we consider deep brain stimulation, and if so, when?,Are there any clinical trials or research studies we can participate in?,How should we monitor for swallowing problems and when should we consider a feeding tube?,What specialists should be part of our care team, and how often should we see them?,Are other family members at risk, and should they be tested?

Common questions about Mitochondrial membrane protein-associated neurodegeneration

What is Mitochondrial membrane protein-associated neurodegeneration?

Mitochondrial membrane protein-associated neurodegeneration (MPAN) is a rare inherited brain disorder that belongs to a group of conditions called neurodegeneration with brain iron accumulation (NBIA). In MPAN, abnormal amounts of iron build up in specific areas of the brain, particularly a region called the basal ganglia, which helps control movement. This iron buildup gradually damages brain cells over time. MPAN is caused by changes (mutations) in the C19orf12 gene, which provides instructions for making a protein found in the membranes of mitochondria — the energy-producing structures ins

How is Mitochondrial membrane protein-associated neurodegeneration inherited?

Mitochondrial membrane protein-associated neurodegeneration follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Which specialists treat Mitochondrial membrane protein-associated neurodegeneration?

2 specialists and care centers treating Mitochondrial membrane protein-associated neurodegeneration are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.