Peroxisomal biogenesis: multiple pathways of protein import.
نویسندگان
چکیده
منابع مشابه
Identification of three distinct peroxisomal protein import defects in patients with peroxisome biogenesis disorders.
Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum's disease, and classical rhizomelic chondrodysplasia punctata are lethal genetic disorders caused by defects in peroxisome biogenesis. We report here a characterization of the peroxisomal matrix protein import capabilities of fibroblasts from 62 of these peroxisome biogenesis disorder patients representing all ten known complem...
متن کاملRecent advances in peroxisomal matrix protein import.
Peroxisomes are essential organelles responsible for many metabolic reactions, such as the oxidation of very long chain and branched fatty acids, D-amino acids and polyamines, as well as the production and turnover of hydrogen peroxide. They comprise a class of organelles called microbodies, including glycosomes, glyoxysomes and Woronin bodies. Dysfunction of human peroxisomes causes severe and...
متن کاملPeroxisomal Protein Import The Paradigm Shifts
oxisomal matrix proteins are imported into the organelle from their site of synthesis on free cytoplasmic ribo-somes. This observation led to the classical model of peroxisome proliferation in which existing peroxisomes Amherst, Massachusetts 01003 increase in size via the direct import of proteins from the cytoplasm, and divide by fission or budding (La-zarow and Fujiki, 1985). As such, the bi...
متن کاملPex14p, a Peroxisomal Membrane Protein Binding Both Receptors of the Two PTS-Dependent Import Pathways
Pex14p, an S. cerevisiae peroxin, is attached to the outer face of the peroxisomal membrane and is a component of the protein import machinery. Pex14p interacts with both the PTS1 and PTS2 receptors. It is the only known peroxisomal membrane protein that binds the PTS2 receptor and might thus mediate the membrane docking event of PTS2-dependent protein import. These results suggest that the two...
متن کاملQuantitative analysis of peroxisomal protein import in vitro.
Protein import into the peroxisome matrix is mediated by peroxisome-targeting signals (PTSs). We have developed a novel, quantitative, in vitro assay for measuring peroxisomal import of PTS1-containing proteins. This enzyme-linked immunosorbent assay-based system utilizes semi-intact human A431 cells or fibroblasts and a biotinylated version of the PTS1-containing import substrate, luciferase. ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 1994
ISSN: 0021-9258
DOI: 10.1016/s0021-9258(18)43771-4