Mitochondrial 4-hydroxyphenylpyruvate dioxygenase-like prote...

created [InstanceEdit:9856699] Stephan, Ralf, 2023-12-22
dbId 9856721
displayName Mitochondrial 4-hydroxyphenylpyruvate dioxygenase-like prote...
schemaClass Summation
text Mitochondrial 4-hydroxyphenylpyruvate dioxygenase-like protein (HPDL) was shown to process 4-hydroxphenylpyruvate (HPPA) to (S)-4-hydroxymandelate (4-HMA). HPDL defects lead to CoQ10 deficiency, and the HPDL product 4-hydroxymandelate apparently is a precursor for the synthesis of 4-hydroxybenzoate, which is a prerequisite for the assembly of the CoQ10 head group (Banh et al., 2021). Because HPDL is a mitochondrial protein, cytosolic HPPA from tyrosine catabolism must be imported by an unknown transport mechanism (Husain et al., 2020; reviewed in Staiano et al., 2023).
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