Disorders related to peroxisomal membranes

Authors
Citation
J. Gartner, Disorders related to peroxisomal membranes, J INH MET D, 23(3), 2000, pp. 264-272
Citations number
41
Categorie Soggetti
Endocrinology, Nutrition & Metabolism
Journal title
JOURNAL OF INHERITED METABOLIC DISEASE
ISSN journal
01418955 → ACNP
Volume
23
Issue
3
Year of publication
2000
Pages
264 - 272
Database
ISI
SICI code
0141-8955(200005)23:3<264:DRTPM>2.0.ZU;2-Q
Abstract
The peroxisome is a ubiquitous, subcellular organelle participating in a di verse array of metabolic pathways. The peroxisomal membrane and its compone nts play a key role in organelle assembly and functions. Disorders related to peroxisomal membranes are the peroxisome biogenesis disorders and X-link ed adrenoleukodystrophy. Identification and functional characterization of these disease genes is proceeding at rapid pace helped immeasurably by work in various yeast model systems. The ultimate goal is to elucidate how the encoded proteins interact to produce apparently normal and functioning pero xisomes. The achievement of this goal will lead to a better understanding o f disease pathogenesis and hopefully open therapeutic options.