CoA synthase protein associated neurodegeneration

CoA synthase protein associated neurodegeneration (CoPAN) is a type of neurodegeneration with brain iron accumulation (NBIA).

Patients typically present with a variety of symptoms including spastic-dystonic paraparesis, Parkinsonism, cognitive impairment, obsessive-compulsive disorder.

Coenzyme A (CoA) synthase (gene: CoASY) is a cofactor involved in enzymatic reactions. Mutations in the CoASY gene leads to brain iron accumulation. 

"Eye of the tiger sign" on T2 MRI (like PKAN) associated with bilateral basal ganglia calcifications.

Article information

rID: 50279
Synonyms or Alternate Spellings:

ADVERTISEMENT: Supporters see fewers/no ads

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.