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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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PANK3
pantothenate kinase 3
Chromosome 5 · 5q34
NCBI Gene: 79646Ensembl: ENSG00000120137.8HGNC: HGNC:19365UniProt: Q9H999
23PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Kinase
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
pantothenate kinase activityATP bindingvitamin bindingprotein homodimerization activitymultiple sclerosisAlzheimer diseaseneurodegenerative diseaseParkinson disease
✦AI Summary

PANK3 (pantothenate kinase 3) catalyzes the phosphorylation of pantothenate to 4'-phosphopantothenate, the first and rate-limiting step of coenzyme A (CoA) biosynthesis 1. PANK3 is the most highly expressed pantothenate kinase isoform in humans 2. The enzyme functions as a homodimer with allosterically coupled active sites that coordinately switch between active and inactive conformations 3. ATP binding induces a conformational change in the amino-terminal nucleotide binding domain to close the active site, while acetyl-CoA provides feedback inhibition, making PANK3 the master regulator of CoA biosynthesis 3. Subcellular fractionation studies demonstrate PANK3 is predominantly cytosolic with broad expression across cell types 2. PANK3 has emerged as a therapeutic target because modulating its activity can affect sensitivity to histone acetyltransferase inhibitors by controlling acetyl-CoA availability 4. Disease relevance includes roles in pantothenate kinase-associated neurodegeneration (PKAN), lipid metabolic disorders, Parkinson's disease, cancer, and insulin resistance 5. Additionally, PANK3 genetic variation associates with brain iron homeostasis, implicating CoA metabolism in neurodegeneration pathways 6.

Sources cited
1
PANK3 catalyzes the first and rate-determining step of CoA biosynthesis from pantothenate; discusses regulation of PANK enzymes in disease
PMID: 38871981
2
PANK3 is the most highly expressed pantothenate kinase; demonstrates PANK3 is predominantly cytosolic with broad expression across cancer cell lines
PMID: 36139163
3
PANK3 functions as a dimer with allosterically coupled active sites; acetyl-CoA feedback inhibition; ATP-induced conformational changes
PMID: 27555321
4
PANK3 regulates CoA biosynthesis and acetyl-CoA levels, affecting sensitivity to histone acetyltransferase inhibitors
PMID: 37127754
5
PANK3 is a therapeutic target in multiple diseases including PKAN, lipid disorders, Parkinson's disease, cancer, and insulin resistance
PMID: 40754168
6
PANK3 genetic variation associates with postmortem brain iron levels, linking CoA metabolism to neurodegeneration
PMID: 39918201
Disease Associationsⓘ20
multiple sclerosisOpen Targets
0.36Weak
Alzheimer diseaseOpen Targets
0.33Weak
lysosomal storage diseaseOpen Targets
0.33Weak
neurodegenerative diseaseOpen Targets
0.33Weak
Parkinson diseaseOpen Targets
0.33Weak
color vision disorderOpen Targets
0.27Weak
Abnormality of the skeletal systemOpen Targets
0.23Weak
aortic diseaseOpen Targets
0.14Weak
adverse effectOpen Targets
0.14Weak
urinary tract obstructionOpen Targets
0.13Weak
sleep apneaOpen Targets
0.10Suggestive
response to stimulusOpen Targets
0.09Suggestive
benign chondrogenic neoplasmOpen Targets
0.09Suggestive
acute tonsillitisOpen Targets
0.08Suggestive
Blackfan-Diamond anemiaOpen Targets
0.05Suggestive
atrial fibrillationOpen Targets
0.05Suggestive
X-linked sideroblastic anemia 1Open Targets
0.04Suggestive
cervical carcinomaOpen Targets
0.03Suggestive
Recurrent infection due to specific granule deficiencyOpen Targets
0.03Suggestive
neutropenia, severe congenital, 10, autosomal recessiveOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
VNN1Protein interaction92%VNN2Protein interaction91%ENPP1Protein interaction90%ENPP3Protein interaction90%PPCDCProtein interaction80%COASYProtein interaction79%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
81%
Heart
65%
Brain
58%
Ovary
43%
Lung
39%
Gene Interaction Network
Click a node to explore
PANK3VNN1VNN2ENPP1ENPP3PPCDCCOASY
PROTEIN STRUCTURE
Preparing viewer…
PDB7UE7 · 1.55 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.75LoF Tolerant
pLIⓘ
0.02Tolerant
Observed/Expected LoF0.49 [0.33–0.75]
RankingsWhere PANK3 stands among ~20K protein-coding genes
  • #13,476of 20,598
    Most Researched23
  • #6,008of 17,882
    Most Constrained (LOEUF)0.75
Genes detectedPANK3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Coenzyme A biosynthesis: mechanisms of regulation, function and disease.
PMID: 38871981
Nat Metab · 2024
1.00
2
Generation and Validation of an Anti-Human PANK3 Mouse Monoclonal Antibody.
PMID: 36139163
Biomolecules · 2022
0.90
3
Acetyl-CoA biosynthesis drives resistance to histone acetyltransferase inhibition.
PMID: 37127754
Nat Chem Biol · 2023
0.80
4
Allosteric Regulation of Mammalian Pantothenate Kinase.
PMID: 27555321
J Biol Chem · 2016
0.70
5
Targeting pantothenate kinases in human diseases: Biochemistry and pharmacotherapy.
PMID: 40754168
Biochem Pharmacol · 2025
0.60