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Field	Query

	Field	Term

General Information:

Id:	7,608
Diseases:	Metabolic Phenylketonuria - [OMIM]
Organism:	Mammalia
Publication type:	review
Reference:	Infante JP and Huszagh VA(2001) Impaired arachidonic (20:4n-6) and docosahexaenoic (22:6n-3) acid synthesis by phenylalanine metabolites as etiological factors in the neuropathology of phenylketonuria Mol. Genet. Metab. 72: 185-198 [PMID: 11243724]

Interaction Information:

Comment	Low levels of of brain arachidonic (20:4n-6) and docosahexaenoic (22:6n-3, DHA) acids are involved in the development of microcephaly and mental retardation of uncontrolled PKU and maternal PKU.
Formal Description Interaction-ID: 76057	drug/chemical compound Arachidonic acid increases_activity of process brain development
Drugbank entries	Show/Hide entries for Arachidonic acid
Drugbank DB04557	Arachidonic Acid not specified PTGS1
Drugbank DB04557	Arachidonic Acid not specified ""
Drugbank DB04557	Arachidonic Acid not specified ""
Drugbank DB04557	Arachidonic Acid not specified PTGS1

Comment	Arachidonic (20:4n-6) and docosahexaenoic (22:6n-3) are synthesized by carnitine-dependent mitochondrial fatty acid desaturases for which alpha-tocopherolquinone is an essential enzyme cofactor. Alpha-tocopherolquinone can be synthesized either de novo or from alpha-tocopherol. The fetus or newborn primarily relies on de novo alpha-TQ synthesis for these mitochondrial fatty acid desaturases because of low maternal transfer of alpha-tocopherol.
Formal Description Interaction-ID: 76058	drug/chemical compound alpha-Tocopherolquinone increases_quantity of drug/chemical compound Arachidonic acid
Drugbank entries	Show/Hide entries for
Drugbank DB04557	Arachidonic Acid not specified PTGS1
Drugbank DB04557	Arachidonic Acid not specified ""
Drugbank DB04557	Arachidonic Acid not specified ""
Drugbank DB04557	Arachidonic Acid not specified PTGS1

Comment	Low levels of of brain arachidonic (20:4n-6) and docosahexaenoic (22:6n-3, DHA) acids are involved in the development of microcephaly and mental retardation of uncontrolled PKU and maternal PKU.
Formal Description Interaction-ID: 76059	drug/chemical compound Docosahexaenoic acid increases_activity of process brain development

Comment	Arachidonic (20:4n-6) and docosahexaenoic (22:6n-3) are synthesized by carnitine-dependent mitochondrial fatty acid desaturases for which alpha-tocopherolquinone is an essential enzyme cofactor. Alpha-tocopherolquinone can be synthesized either de novo or from alpha-tocopherol. The fetus or newborn primarily relies on de novo alpha-TQ synthesis for these mitochondrial fatty acid desaturases because of low maternal transfer of alpha-tocopherol.
Formal Description Interaction-ID: 76060	drug/chemical compound alpha-Tocopherolquinone increases_quantity of drug/chemical compound Docosahexaenoic acid

Comment	The major catabolic products of excess phenylalanine, phenylpyruvate and phenyllactate, are proposed to inhibit alpha-TQ synthesis by inhibiting 4-hydroxyphenylpyruvate dioxygenase.
Formal Description Interaction-ID: 76061	phenotype Hyperphenylalaninemia decreases_quantity of drug/chemical compound alpha-Tocopherol by inhibiting 4-hydroxyphenylpyruvate dioxygenase

Comment	Arachidonic (20:4n-6) and docosahexaenoic (22:6n-3) are synthesized by carnitine-dependent mitochondrial fatty acid desaturases for which alpha-tocopherolquinone is an essential enzyme cofactor. Alpha-tocopherolquinone can be synthesized either de novo or from alpha-tocopherol. The fetus or newborn primarily relies on de novo alpha-TQ synthesis for these mitochondrial fatty acid desaturases because of low maternal transfer of alpha-tocopherol.
Formal Description Interaction-ID: 76062	drug/chemical compound alpha-Tocopherol increases_quantity of drug/chemical compound alpha-Tocopherolquinone

Comment	The major catabolic products of excess phenylalanine, phenylpyruvate and phenyllactate, are proposed to inhibit alpha-TQ synthesis by inhibiting 4-hydroxyphenylpyruvate dioxygenase.
Formal Description Interaction-ID: 76063	drug/chemical compound Phenylpyruvate decreases_quantity of drug/chemical compound alpha-Tocopherol by inhibiting 4-hydroxyphenylpyruvate dioxygenase

Comment	The major catabolic products of excess phenylalanine, phenylpyruvate and phenyllactate, are proposed to inhibit alpha-TQ synthesis by inhibiting 4-hydroxyphenylpyruvate dioxygenase.
Formal Description Interaction-ID: 76064	drug/chemical compound Phenyllactate decreases_quantity of drug/chemical compound alpha-Tocopherol by inhibiting 4-hydroxyphenylpyruvate dioxygenase

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