Biomaterial Database

Androgens and fatty acid metabolism in X-linked Adrenoleukodystrophy. 2002

A Petroni

Department of Pharmacological Sciences, University of Milan, Milan, Italy. Anna.Petroni@unimi.it

X-Adrenoleukodystrophy (X-ALD) is an inherited pathology characterized by very long-chain fatty acid accumulation in plasma as well as in different tissues. The nervous system, the adrenal cortex and the testis are primarily affected. Steroid metabolism which occurs in the adrenal cortex and in the testis might be severely impaired. We have hypothesized that steroids, in particular the androgens, might have a role in this pathology. We have demonstrated that the testosterone metabolite dihydrotestosterone (DHT) and 5alpha-androstan-3alpha,17beta-diol (3alpha-diol), but not testosterone itself, when incubated in skin fibroblasts obtained from patients affected by X-ALD, significantly reduced the abnormal accumulation of very long-chain fatty acids.

UI	MeSH Term	Description	Entries
D005227	Fatty Acids	Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed)	Aliphatic Acid,Esterified Fatty Acid,Fatty Acid,Fatty Acids, Esterified,Fatty Acids, Saturated,Saturated Fatty Acid,Aliphatic Acids,Acid, Aliphatic,Acid, Esterified Fatty,Acid, Saturated Fatty,Esterified Fatty Acids,Fatty Acid, Esterified,Fatty Acid, Saturated,Saturated Fatty Acids
D005347	Fibroblasts	Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules.	Fibroblast
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000326	Adrenoleukodystrophy	An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS).	Adrenomyeloneuropathy,Schilder-Addison Complex,X-Linked Adrenoleukodystrophy,ALD (Adrenoleukodystrophy),Addison Disease and Cerebral Sclerosis,Bronze Schilder Disease,Melanodermic Leukodystrophy,Siemerling-Creutzfeldt Disease,X-ALD,X-ALD (X-Linked Adrenoleukodystrophy),Adrenoleukodystrophy, X-Linked,Leukodystrophies, Melanodermic,Leukodystrophy, Melanodermic,Schilder Addison Complex,Siemerling Creutzfeldt Disease,X ALD,X ALD (X Linked Adrenoleukodystrophy),X Linked Adrenoleukodystrophy
D000728	Androgens	Compounds that interact with ANDROGEN RECEPTORS in target tissues to bring about the effects similar to those of TESTOSTERONE. Depending on the target tissues, androgenic effects can be on SEX DIFFERENTIATION; male reproductive organs, SPERMATOGENESIS; secondary male SEX CHARACTERISTICS; LIBIDO; development of muscle mass, strength, and power.	Androgen,Androgen Receptor Agonist,Androgen Effect,Androgen Effects,Androgen Receptor Agonists,Androgenic Agents,Androgenic Compounds,Agents, Androgenic,Agonist, Androgen Receptor,Agonists, Androgen Receptor,Compounds, Androgenic,Effect, Androgen,Effects, Androgen,Receptor Agonist, Androgen,Receptor Agonists, Androgen
D012867	Skin	The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D013196	Dihydrotestosterone	A potent androgenic metabolite of TESTOSTERONE. It is produced by the action of the enzyme 3-OXO-5-ALPHA-STEROID 4-DEHYDROGENASE.	5 alpha-Dihydrotestosterone,Androstanolone,Stanolone,17 beta-Hydroxy-5 beta-Androstan-3-One,17beta-Hydroxy-5alpha-Androstan-3-One,5 beta-Dihydrotestosterone,5-alpha Dihydrotestosterone,5-alpha-DHT,Anaprotin,Andractim,Dihydroepitestosterone,Gelovit,17 beta Hydroxy 5 beta Androstan 3 One,17beta Hydroxy 5alpha Androstan 3 One,5 alpha DHT,5 alpha Dihydrotestosterone,5 beta Dihydrotestosterone,Dihydrotestosterone, 5-alpha,beta-Hydroxy-5 beta-Androstan-3-One, 17
D013739	Testosterone	A potent androgenic steroid and major product secreted by the LEYDIG CELLS of the TESTIS. Its production is stimulated by LUTEINIZING HORMONE from the PITUITARY GLAND. In turn, testosterone exerts feedback control of the pituitary LH and FSH secretion. Depending on the tissues, testosterone can be further converted to DIHYDROTESTOSTERONE or ESTRADIOL.	17-beta-Hydroxy-4-Androsten-3-one,17-beta-Hydroxy-8 alpha-4-Androsten-3-one,8-Isotestosterone,AndroGel,Androderm,Andropatch,Androtop,Histerone,Sterotate,Sustanon,Testim,Testoderm,Testolin,Testopel,Testosterone Sulfate,17 beta Hydroxy 4 Androsten 3 one,17 beta Hydroxy 8 alpha 4 Androsten 3 one,8 Isotestosterone
D015113	Androstane-3,17-diol	The unspecified form of the steroid, normally a major metabolite of TESTOSTERONE with androgenic activity. It has been implicated as a regulator of gonadotropin secretion.	5 Androstane 3,17 diol,5 alpha-Androstane-3 alpha,17 beta-diol,5 alpha-Androstane-3 beta,17 alpha-diol,5 alpha-Androstane-3 beta,17 beta-diol,5 alpha-Androstane-3alpha,17 beta-diol,5 beta-Androstane-3 alpha,17 beta-diol,5-Androstane-3,17-diol,5alpha-Androstane-3beta,17alpha-diol,5 alpha Androstane 3 alpha,17 beta diol,5 alpha Androstane 3 beta,17 alpha diol,5 alpha Androstane 3 beta,17 beta diol,5 alpha Androstane 3alpha,17 beta diol,5 beta Androstane 3 alpha,17 beta diol,5alpha Androstane 3beta,17alpha diol,Androstane 3,17 diol