Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

Official Name

pantothenate kinase

Name from literature

pantothenate kinase

Pathway from literature

coenzyme A ( CoA ) biosynthetic pathway

Pathway from KEGG

Metabolism of Cofactors and Vitamins; Pantothenate and CoA biosynthesis; map00770


Human (9606)

Genome localization

20p13[80025 ], 1p36.32[55229 ], 5q34[79646 ], 10q23.31[53354 ],

Rate-limiting Description

"Pantothenate kinase (PanK) is thought to catalyze the first rate-limiting step in CoA biosynthesis." (14523052)

Regulatory Information

Upstream transcription factor


Regulatory type


transcriptional factor;PPARalpha transcription factor(5465)

"PPARalpha transcription factor as a major factor governing hepatic CoA levels by specific modulation of PANK1alpha gene expression" (14523052)


"PanK4 interacts with Pkm2 and thereby may modulate the glucose metabolism through regulating the activity of Pkm2." (16132722)







Gene ontology

Gene ontology

GO:0005739 (C) mitochondrion [Q9BZ23 ];
GO:0004594 (F) pantothenate kinase activity [Q9H999, Q9BZ23, Q8TE04, Q9NVE7 ];
GO:0005737 (C) cytoplasm [Q9H999, Q9NVE7 ];
GO:0015937 (P) coenzyme A biosynthetic process [Q9H999, Q9BZ23, Q8TE04, Q9NVE7 ];
GO:0005829 (C) cytosol [Q8TE04 ];
GO:0005524 (F) ATP binding [Q9H999, Q9BZ23, Q8TE04, Q9NVE7 ];

Tissue expression

Tissue From HPRD

Kidney [07312, 07549, 05857, 07313 ];
Ubiquitous [05857 ];
Heart [07312, 07549, 05857, 07313 ];
Lung [05857, 07313 ];
Testis [07549 ];
Pancreas [07312, 05857, 07313 ];
Skeletal muscle [07549 ];
Liver [07312, 07549, 05857, 07313 ];
Placenta [07312, 05857, 07313 ];
Retina [05857 ];
Brain [07312, 07549, 05857, 07313 ];
Muscle [07312, 05857, 07313 ];

Tissue specificity

Highly expressed in the liver [Q9H999 ];

Ubiquitously expressed with higher expression in the muscle [Q9NVE7 ];

Ubiquitous [Q9BZ23 ];

Isoform 1 is expressed at high levels in brain, heart, kidney, liver, skeletal muscle and testis. Isoform 2 is detected at much lower levels in kidney, liver, brain and testis and is not detected in heart or skeletal muscle [Q8TE04 ];

Subcellular localization


mitochondrion [Q9BZ23 ];

cytoplasm [Q9H999, Q9BZ23, Q8TE04, Q9NVE7 ];

Disease relevance


Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP) [MIM:607236]. HARP is a rare syndrome with many clinical similarities to PKAN [Q9BZ23 ];

Defects in PANK2 are the cause of pantothenate kinase-associated neurodegeneration (PKAN) [MIM:234200];
formerly known as Hallervorden-Spatz syndrome (HSS). PKAN is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain. Clinical features include extrapyramidal dysfunction, and a relentlessly progressive course. Atypical PKAN is diagnosed in individuals who may not fit with the diagnostic criteria of PKAN yet have radiographic or pathologic evidence of increased basal ganglia iron [Q9BZ23 ];



Q8TE04; Q9BZ23; Q9H999; Q9NVE7

Entrez Gene

53354; 55229; 79646; 80025


05857; 07312; 07549; 07313

  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min