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Pantothenate Kinase 2 (PANK2)
- Target
- PANK2
- Molecular classification
- Enzyme, Kinase
01
Overview
Pantothenate kinase 2 (PANK2) is a mitochondrial enzyme that catalyzes the first committed step in coenzyme A (CoA) biosynthesis. It is critical for cellular metabolism, particularly energy production from carbohydrates, fats, and amino acids. Mutations in PANK2 cause pantothenate kinase-associated neurodegeneration (PKAN), a progressive neurodegenerative disorder characterized by abnormal iron accumulation in the brain.
- Other names
- NBIA1PANK2_HUMANPantothenic acid kinasePantothenate kinase 2 (Hallervorden-Spatz syndrome)
02
Mechanism of action
Catalyzes the phosphorylation of pantothenic acid, the first committed step in coenzyme A biosynthesis.
03
Biological functions
Coenzyme A biosynthesisRegulation of cellular metabolism
04
Disease associations
Pantothenate kinase-associated neurodegeneration (PKAN)HARP syndrome
05
Safety considerations
Mutations lead to neurodegeneration and iron accumulation in the brain.
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