Premium
Disorders related to peroxisomal membranes
Author(s) -
Gärtner J.
Publication year - 2000
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1005636113499
Subject(s) - peroxisome , peroxisomal disorder , adrenoleukodystrophy , organelle , biogenesis , biology , zellweger syndrome , computational biology , disease , identification (biology) , human genetics , organelle biogenesis , microbiology and biotechnology , bioinformatics , gene , genetics , medicine , pathology , botany
The peroxisome is a ubiquitous, subcellular organelle participating in a diverse array of metabolic pathways. The peroxisomal membrane and its components play a key role in organelle assembly and functions. Disorders related to peroxisomal membranes are the peroxisome biogenesis disorders and X‐linked 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 peroxisomes. The achievement of this goal will lead to a better understanding of disease pathogenesis and hopefully open therapeutic options.