Biomaterial Database

Disturbances of valine metabolism in patients with peroxisomal biogenesis disorders. 2003

Francois J M Eyskens, and Marc Lefevere

Provincial Centre for Metabolic Disorders (PCMA), Antwerp, Belgium. Francois.Eyskens@pcma.provant.be

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014631	Valerates	Derivatives of valeric acid, including its salts and esters.	Pentanoate,Pentanoates,Valerate
D014633	Valine	A branched-chain essential amino acid that has stimulant activity. It promotes muscle growth and tissue repair. It is a precursor in the penicillin biosynthetic pathway.	L-Valine,L Valine
D018901	Peroxisomal Disorders	A heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional PEROXISOMES. Peroxisomal enzymatic abnormalities may be single or multiple. Biosynthetic peroxisomal pathways are compromised, including the ability to synthesize ether lipids and to oxidize long-chain fatty acid precursors. Diseases in this category include ZELLWEGER SYNDROME; INFANTILE REFSUM DISEASE; rhizomelic chondrodysplasia (CHONDRODYSPLASIA PUNCTATA, RHIZOMELIC); hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and ADRENOLEUKODYSTROPHY (X-linked). Neurologic dysfunction is a prominent feature of most peroxisomal disorders.	Adrenoleukodystrophy, Neonatal,Hyperpipecolic Acidemia,Adrenoleukodystrophy, Autosomal Neonatal Form,Adrenoleukodystrophy, Autosomal, Neonatal Form,Hyperpipecolatemia,Neonatal Adrenoleukodystrophy,Peroxisomal Dysfunction, General,Peroxisomal Dysfunction, Multiple,Peroxisomal Dysfunction, Single,Acidemia, Hyperpipecolic,Acidemias, Hyperpipecolic,Adrenoleukodystrophies, Neonatal,Dysfunction, General Peroxisomal,Dysfunction, Multiple Peroxisomal,Dysfunction, Single Peroxisomal,Dysfunctions, General Peroxisomal,Dysfunctions, Multiple Peroxisomal,Dysfunctions, Single Peroxisomal,General Peroxisomal Dysfunction,General Peroxisomal Dysfunctions,Hyperpipecolic Acidemias,Multiple Peroxisomal Dysfunction,Multiple Peroxisomal Dysfunctions,Neonatal Adrenoleukodystrophies,Peroxisomal Disorder,Peroxisomal Dysfunctions, General,Peroxisomal Dysfunctions, Multiple,Peroxisomal Dysfunctions, Single,Single Peroxisomal Dysfunction,Single Peroxisomal Dysfunctions