2.7.1.33: pantothenate kinase

This is an abbreviated version!
For detailed information about pantothenate kinase, go to the full flat file.

Reaction

Synonyms

4-phosphopantoate, BaPanK, Cab1, Cab1p, CoaA, coaW, CoaX, D-pantothenate kinase, EhPAnK, fumble, hPanK, hPanK1, hPANK2, hPanK3, hPanK4, HpPanK-III, HsPANK3, HsPANK4, kinase, pantothenate (phosphorylating), More, mPank, mPank1, mPanK2, mPanK3, MtCoaA, MtPanK, PAK, PanK, PanK-III, PanK1, PanK1alpha, PanK1b, PanK2, PanK3, PanK4, PanKBa, pantothenate kinase, pantothenate kinase 1, pantothenate kinase 2, pantothenate kinase 3, pantothenate kinase 4, pantothenate kinase-2, pantothenic acid kinase, PfPanK, Pfpank1, PoK, rPanK4, Rts protein , Rv1092c, TK2141, TmPanK-III, type II pantothenate kinase, type III pantothenate kinase

ECTree

     2 Transferases

         2.7 Transferring phosphorus-containing groups

             2.7.1 Phosphotransferases with an alcohol group as acceptor

                2.7.1.33 pantothenate kinase

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Results	in table
20	Activating Compound
42099	AA Sequence
16	Application
1	CAS Registry Number
58	Cloned(Commentary)
29	Cofactor
23	Crystallization (Commentary)
732	Disease/ Diagnostics
52	General Information
7	General Stability
85	IC50 Value
335	Inhibitors
41	kcat/KM [mM/s]
19	Ki Value [mM]
104	KM Value [mM]
42	Localization
65	Metals/Ions
20	Molecular Weight [Da]
74	Natural Substrates/ Products (Substrates)
698	Organism
7	Pathway
190	PDB ID
21	pH Optimum
2	pH Range
4	pH Stability
2	pI Value
1	Posttranslational Modification
82	Protein Variants
45	Purification (Commentary)
3	Reaction
1	Reaction Type
135	Reference
49	Source Tissue
10	Specific Activity [micromol/min/mg]
6	Storage Stability
215	Substrates and Products (Substrate)
30	Subunits
196	Synonyms
1	Systematic Name
19	Temperature Optimum [°C]
3	Temperature Range [°C]
13	Temperature Stability [°C]
58	Turnover Number [1/s]

Application

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Application on EC 2.7.1.33 - pantothenate kinase

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APPLICATION

ORGANISM

UNIPROT

COMMENTARY

LITERATURE

drug development

Entamoeba histolytica

B1N2P3

since Entamoeba histolytica PanK (EhPanK) is physiologically important and sufficiently divergent from its human orthologs, this enzyme represents an attractive target for the development of novel anti-amebic chemotherapies

759375

medicine

14 entries

synthesis

Enterococcus faecalis

Q839J7

synthesis of (carboxyl-18O)phosphopantothenate

702284

medicine

Homo sapiens

-

a young girl with early onset pantothenate kinase-associated neurodegeneration whose initial clinical manifestation is ataxia at the age of 2.5 years and refractory severe dystonia resulting in essentially complete loss of motor control. She has a mutation in PANK2 gene consisting of an amino acid change of alanine to valine in exon 5 (A382V). After Globus Pallidus deep brain stimulation at the age of 11 years, the patient regains useful motor function and speech with a marked decrease in the severity of the dystonia

686546

medicine

Homo sapiens

-

neurodegeneration with brain iron accumulation is a heterogenous group of disorder. One group of patiens bears mutations in the gene encoding pantothenate kinase 2

684227

medicine

Homo sapiens

-

pallidal stimulation for dystonia in pantothenate kinase associated neurodegeneration

689335

medicine

Homo sapiens

-

pantothenate kinase associated neurodegeneration is an autosomal recessive disorder characterized by dystonia, parkinsonism, and iron accumulation in the brain. Many patients have a mutation in the gene encoding pantothenate kinase 2 which is a key regulator enzyme in the biosynthesis of coenzyme A

690019

medicine

Homo sapiens

-

pantothenate kinase-associated neurodegeneration (formerly Hallervorden-Spatz syndrome), the most prevalent form of neurodegeneration with brain iron accumulation, is a rare degenerative brain disease characterised by predominantly extrapyramidal dysfunction resulting from mutations in the PANK2 (pantothenate kinase 2) gene. A novel missense mutation (P354L) in exon 4 of the PANK2 gene is identified in an adolescent with classic pantothenate kinase-associated neurodegeneration. DNA-based diagnosis of pantothenate kinase-associated neurodegeneration plays a key role in determination, and can make the diagnosis more simply, directly, and economically because it obviates the need for unnecessary biochemical tests. Once pantothenate kinase-associated neurodegeneration-like symptoms are identified, mutation analysis and target screening for the family of the proband can provide efficient and accurate evidence of pantothenate kinase-associated neurodegeneration inheritance

687016

medicine

Homo sapiens

-

pantothenate kinase-associated neurodegeneration is a neurodegenerative condition with a broad phenotypic spectrum

688202

medicine

Homo sapiens

-

pantothenate kinase-associated neurodegeneration is a progressive neurodegenerative disorder with autosomal recessive inheritance. The major symptoms of PKAN include the onset before the age of 20 years, progressive pyramidal and extrapyramidal signs, retinitis pigmentosa, optic atrophy, dementia, and iron depositions in the globus pallidus. Identification of mutations of PANK2 gene in patients with proven molecular diagnosis of pantothenate kinase-associated neurodegeneration

687943

medicine

Homo sapiens

-

pantothenate kinase-associated neurodegeneration is an autosomal-recessive disorder associated with the accumulation of iron in the basal ganglia. The disease presents with dystonia, rigidity, and gait impairment, leading to restriction of activities and loss of ambulation. The disorder is caused by defective iron metabolism associated with mutations in the PANK2 gene, which codes for the pantothenate kinase enzyme. A mutation screen conducted in two siblings to establish a molecular diagnosis of the disease and a genetic test for the family is reported

689334

medicine

Homo sapiens

Q9BZ23

pantothenate-kinase-associated neurodegeneration (PKAN) is caused by mutations of the pantothenate kinase (PANK2) on chromosome 20p13. PKAN is characterized clinically by extrapyramidal symptoms (in 98% of cases), in particular, generalized dystonia with oromandibular involvement, and parkinsonism-spasticity (25%), behavioral changes followed by dementia (29%), and pigmentary retinal degeneration. The mean age at onset is between 3 and 4 years

689128

medicine

Homo sapiens

Q9BZ23

pantothenate-kinase-associated neurodegeneration is caused by mutations of the pantothenate kinase gene. Pantothenate-kinase-associated neurodegeneration is characterized clinically by extrapyramidal symptoms (in 98% of cases), in particular, generalized dystonia with oromandibular involvement, and parkinsonism-spasticity (25%), behavioral changes followed by dementia (29%), and pigmentary retinal degeneration. The mean age at onset is between 3 and 4 years

689128

medicine

Mus musculus

-

pantothenic acid deprivation provides a useful phenocopy for pantothenate kinase associated neurodegeneration (formerly called Hallervorden-Spatz syndrome) and allows us to test pharmacological and other interventional strategies in the treatment of this devastating disease

688176

medicine

Homo sapiens

-

patient with pantothenate kinase-associated neurodegeneration whose dystonia and freezing of gait respond dramatically to anticholinergic treatment

689108

medicine

Homo sapiens

-

the cognitive deterioration in pantothenate kinase-associated neurodegeneration dystonia has been overemphasised and many of the apparent disabilities are more a consequence of the dystonia

684736

medicine

Mus musculus

-

the enzyme is a suitable target for therapeutic intervention in metabolic disorders that feature hyperglycemia and hypertriglyceridemia

723517