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Literature summary for 2.7.1.33 extracted from
- Altered neuronal mitochondrial coenzyme A synthesis in neurodegeneration with brain iron accumulation caused by abnormal processing, stability, and catalytic activity of mutant pantothenate kinase 2 (2005), J. Neurosci., 25, 689-698.
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| expression of wild-type His/myc-tagged isozyme PanK2 and of mutant enzymes in mitochondria of QBI 293 cells | Homo sapiens |
Protein Variants
| Protein Variants | Comment | Organism |
|---|---|---|
| E134G | naturally occurring disease-related point mutation which leads to reduced enzyme activity, and altered processing and stability of the mutant PanK2, reconstruction by site-sirected mutagenesis | Homo sapiens |
| G219V | naturally occurring disease-related point mutation which leads to reduced enzyme activity, and altered processing and stability of the mutant PanK2, reconstruction by site-sirected mutagenesis | Homo sapiens |
| G521R | naturally occurring disease-related point mutation which leads to reduced enzyme activity, and altered processing and stability of the mutant PanK2, reconstruction by site-sirected mutagenesis | Homo sapiens |
| additional information | identification of naturally occuring pantothenate kinase 2 mutant in patients with neurodegenerative disease in brain with iron accumulation, formerly termed Hallervorden-Spatz disease, identification of other disease related point mutations which lead to reduced enzyme activity, mutations alter processing, stability, and catalytic activity of the mutant PanK2 | Homo sapiens |
| S471N | naturally occurring disease-related point mutation which leads to reduced enzyme activity, and altered processing and stability of the mutant PanK2, reconstruction by site-sirected mutagenesis | Homo sapiens |
| T234A | naturally occurring disease-related point mutation which leads to reduced enzyme activity, and altered processing and stability of the mutant PanK2, reconstruction by site-sirected mutagenesis | Homo sapiens |
| T528M | naturally occurring disease-related point mutation which leads to reduced enzyme activity, and altered processing and stability of the mutant PanK2, reconstruction by site-sirected mutagenesis | Homo sapiens |
Inhibitors
| Inhibitors | Comment | Organism | Structure |
|---|---|---|---|
| additional information | feedback inhibition by acyl-CoAs | Homo sapiens |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| dendrite | - |
Homo sapiens | 30425 | - |
| mitochondrion | several isoforms of brain PanK2 | Homo sapiens | 5739 | - |
| additional information | multiple subcellular distribution of multiple enzyme forms | Homo sapiens | - |
- |
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 48000 | - |
x * 48000, recombinant His/myc-tagged isozyme PanK2, SDS-PAGE, x * 63000, recombinant unprocessed PanK2, SDS-PAGE | Homo sapiens |
| 63000 | - |
x * 48000, recombinant His/myc-tagged isozyme PanK2, SDS-PAGE, x * 63000, recombinant unprocessed PanK2, SDS-PAGE | Homo sapiens |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| ATP + (R)-pantothenate | Homo sapiens | first step in coenzyme A biosynthesis | ADP + (R)-4'-phosphopantothenate | - |
? |  |
| additional information | Homo sapiens | naturally occuring pantothenate kinase 2 mutant in patients with neurodegenerative disease in brain with iron accumulation, formerly termed Hallervorden-Spatz disease | ? | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
multiple genes, multiple enzyme forms | - |
Posttranslational Modification
| Posttranslational Modification | Comment | Organism |
|---|---|---|
| proteolytic modification | several isoforms of PanK2 are produced by sequential proteolytic cleavage, identification of cleavage sites for the mitochondrial processing peptidase, overview | Homo sapiens |
Purification (Commentary)
| Purification (Comment) | Organism |
|---|---|
| recombinant His/myc-tagged isozyme PanK2 from mitochondria of QBI 293 cells by ion exchange and protein A affinity chromatography and gel filtration | Homo sapiens |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| brain | subtissue distribution of several isoforms of PanK2, overview | Homo sapiens | - |
| additional information | naturally occuring pantothenate kinase 2 mutant in patients with neurodegenerative disease in brain with iron accumulation, formerly termed Hallervorden-Spatz disease | Homo sapiens | - |
| neuron | - |
Homo sapiens | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| ATP + (R)-pantothenate | - |
Homo sapiens | ADP + (R)-4'-phosphopantothenate | - |
? | |
| ATP + (R)-pantothenate | first step in coenzyme A biosynthesis | Homo sapiens | ADP + (R)-4'-phosphopantothenate | - |
? | |
| additional information | naturally occuring pantothenate kinase 2 mutant in patients with neurodegenerative disease in brain with iron accumulation, formerly termed Hallervorden-Spatz disease | Homo sapiens | ? | - |
? | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| ? | x * 48000, recombinant His/myc-tagged isozyme PanK2, SDS-PAGE, x * 63000, recombinant unprocessed PanK2, SDS-PAGE | Homo sapiens |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| PanK2 | - |
Homo sapiens |
| pantothenate kinase 2 | - |
Homo sapiens |
Cofactor
| Cofactor | Comment | Organism | Structure |
|---|---|---|---|
| ATP | - |
Homo sapiens | |
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Release 2023.1 (January 2023)
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