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Literature summary for 2.7.1.33 extracted from

  • Zhang, Y.M.; Rock, C.O.; Jackowski, S.
    Biochemical properties of human pantothenate kinase 2 isoforms and mutations linked to pantothenate kinase-associated neurodegeneration (2006), J. Biol. Chem., 281, 107-114.
    View publication on PubMed
Search for more literature for 2.7.1.33

Cloned(Commentary)

Cloned (Comment) Organism
splice variant PanK2, expression of active PanK2 isozymes iPanK2, spPanK2, mPanK2 in HEK293T cells, in vitro transcription and translation of PanK2 Homo sapiens

Protein Variants

Protein Variants Comment Organism
A509V naturally occurring mutation, early onset in patients, 105% activity compared to the wild-type enzyme Homo sapiens
G219V naturally occurring mutation, early and late onset in patients, 0.4% activity compared to the wild-type enzyme Homo sapiens
G521R the splice variant PanK2 naturally contains mutation which is associated with neurodegenerative disease in brain, early and late onset in patients, less than 0.2% activity compared to the wild-type enzyme Homo sapiens
K224A site-directed mutagenesis, less than 0.2% activity compared to the wild-type enzyme Homo sapiens
additional information several natural mutants with frame shifts show no activity, identification of mutants with mutations which introduce stop codons, overview Homo sapiens
N404I naturally occurring mutation, early and late onset in patients, 83% activity compared to the wild-type enzyme Homo sapiens
N500I naturally occurring mutation, early onset in patients, 3.9% activity compared to the wild-type enzyme Homo sapiens
R264W naturally occurring mutation, early onset in patients, 58% activity compared to the wild-type enzyme Homo sapiens
R286C naturally occurring mutation, early and late onset in patients, 176% activity compared to the wild-type enzyme Homo sapiens
R532W naturally occurring mutation, early onset in patients, 95% activity compared to the wild-type enzyme Homo sapiens
S351P naturally occurring mutation, early and late onset in patients, 78% activity compared to the wild-type enzyme Homo sapiens
T234A naturally occurring mutation, early and late onset in patients, 112% activity compared to the wild-type enzyme Homo sapiens
T327I naturally occurring mutation, early onset in patients, 91% activity compared to the wild-type enzyme Homo sapiens
T528M naturally occurring mutation, early and late onset in patients, 146% activity compared to the wild-type enzyme Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
CoA feedback inhibition, inhibition kinetics of recombinant isozyme mPanK2 Homo sapiens
CoA esters feedback inhibition, inhibition kinetics of recombinant isozyme mPanK2 Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
additional information
-
 additional information kinetics of recombinant isozyme mPanK2 Homo sapiens
0.009
-
 (R)-pantothenate recombinant isozyme iPanK2, pH 7.5, 37°C Homo sapiens
0.025
-
 (R)-pantothenate recombinant isozyme mPanK2, pH 7.5, 37°C Homo sapiens
0.064
-
 ATP recombinant isozyme mPanK2, pH 7.5, 37°C Homo sapiens
0.068
-
 ATP recombinant isozyme iPanK2, pH 7.5, 37°C Homo sapiens

Metals/Ions

Metals/Ions Comment Organism Structure
Mg2+
-
 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
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
 splice variant pantothenate kinase 2, i.e. PanK2, contains mutation G521R, several isozymes e.g. iPanK2, spPanK2, mPanK2, overview
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + (R)-pantothenate first step in coenzyme A biosynthesis Homo sapiens ADP + (R)-4'-phosphopantothenate
-
 ?
ATP + (R)-pantothenate the phosphate binding loop of isozyme mPanK2 is the N-terminal motif DIGGT(S)XXK Homo sapiens ADP + (R)-4'-phosphopantothenate
-
 ?

Subunits

Subunits Comment Organism
dimer molecular mass, 30.8-61.2 kDa, of diverse protein variants expressed from different plasmids in different systems, SDS-PAGE and gel filtration, overview Homo sapiens

Synonyms

Synonyms Comment Organism
PanK2
-
 Homo sapiens
pantothenate kinase 2
-
 Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
 assay at Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7.5
-
 assay at Homo sapiens

Cofactor

Cofactor Comment Organism Structure
ATP
-
 Homo sapiens