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Literature summary for 2.3.1.21 extracted from

  • Yao, M.; Cai, M.; Yao, D.; Xu, X.; Yang, R.; Li, Y.; Zhang, Y.; Kido, H.; Yao, D.
    Abbreviated half-lives and impaired fuel utilization in carnitine palmitoyltransferase II variant fibroblasts (2015), PLoS ONE, 10, e0119936.
    View publication on PubMedView publication on EuropePMC

Protein Variants

Protein Variants Comment Organism
F352C/V368I the mutant shows thermal instability, reduced residual enzyme activities and a short half-life compared to the wild type enzyme Homo sapiens
V368I the mutant shows thermal instability, reduced residual enzyme activities and a short half-life compared to the wild type enzyme Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
palmitoyl-CoA + L-carnitine Homo sapiens
-
CoA + L-palmitoylcarnitine
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens P23786
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
Homo sapiens
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
0.00018
-
mutant enzyme F352C/V368I, at 30°C, pH not specified in the publication Homo sapiens
0.00027
-
mutant enzyme V368I, at 30°C, pH not specified in the publication Homo sapiens
0.00028
-
wild type enzyme, at 30°C, pH not specified in the publication Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
palmitoyl-CoA + L-carnitine
-
Homo sapiens CoA + L-palmitoylcarnitine
-
?

Subunits

Subunits Comment Organism
homotetramer
-
Homo sapiens

Synonyms

Synonyms Comment Organism
carnitine palmitoyltransferase II
-
Homo sapiens
CPT II
-
Homo sapiens

General Information

General Information Comment Organism
malfunction enzyme deficiency leads to a significant decrease in fatty acid beta-oxidation and adenosine triphosphate generation, combined with a reduced mitochondrial membrane potential, resulting in cellular apoptosis Homo sapiens