Rhizomelic chondrodysplasia punctata is a peroxisomal protein targeting disease caused by a non-functional PTS2 receptor

The Metabolic and Molecular Bases of Inherited Disease

1995 • 12,086 citations

The ancient regulatory-protein family of WD-repeat proteins

1994 • 1,478 citations

A novel, cleavable peroxisomal targeting signal at the amino-terminus of the rat 3-ketoacyl-CoA thiolase.

1991 • 629 citations

Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders

1995 • 430 citations

A unified nomenclature for peroxisome biogenesis factors.

1996 • 413 citations

Protein Import into Peroxisomes and Biogenesis of the Organelle

1993 • 411 citations

Multiple PEX genes are required for proper subcellular distribution and stability of Pex5p, the PTS1 receptor: evidence that PTS1 protein import is mediated by a cycling receptor.

1996 • 319 citations

PAS7 encodes a novel yeast member of the WD-40 protein family essential for import of 3-oxoacyl-CoA thiolase, a PTS2-containing protein, into peroxisomes.

1994 • 295 citations

Restoration by a 35K membrane protein of peroxisome assembly in a peroxisome-deficient mammalian cell mutant

1991 • 250 citations

Pex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptor.

1996 • 248 citations

The pas8 mutant of Pichia pastoris exhibits the peroxisomal protein import deficiencies of Zellweger syndrome cells--the PAS8 protein binds to the COOH-terminal tripeptide peroxisomal targeting signal, and is a member of the TPR protein family [published erratum appears in J Cell Biol 1993 Sep;122(5):following 1143]

1993 • 246 citations

The WD‐40 repeat

1992 • 241 citations

PAS10 is a tetratricopeptide-repeat protein that is essential for the import of most matrix proteins into peroxisomes of Saccharomyces cerevisiae.

1993 • 225 citations

Identification of Pex13p a peroxisomal membrane receptor for the PTS1 recognition factor.

1996 • 225 citations

The SH3 domain of the Saccharomyces cerevisiae peroxisomal membrane protein Pex13p functions as a docking site for Pex5p, a mobile receptor for the import PTS1-containing proteins.

1996 • 224 citations

Import of proteins into peroxisomes and other microbodies

1992 • 210 citations

Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders.

1995 • 187 citations

The peroxisome biogenesis disorder group 4 gene, PXAAA1, encodes a cytoplasmic ATPase required for stability of the PTS1 receptor.

1996 • 184 citations

The import receptor for the peroxisomal targeting signal 2 (PTS2) in Saccharomyces cerevisiae is encoded by the PAS7 gene.

1996 • 181 citations

Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the α-subunit of the mitochondrial trifunctional protein

1994 • 158 citations

Analysis of the Carboxyl-terminal Peroxisomal Targeting Signal 1 in a Homologous Context in Saccharomyces cerevisiae

1996 • 158 citations

PEB1 (PAS7) in Saccharomyces cerevisiae encodes a hydrophilic, intra-peroxisomal protein that is a member of the WD repeat family and is essential for the import of thiolase into peroxisomes.

1995 • 140 citations

The Hansenula polymorpha PER3 Gene Is Essential for the Import of PTS1 Proteins into the Peroxisomal Matrix

1995 • 130 citations

Human dihydroxyacetonephosphate acyltransferase deficiency: A new peroxisomal disorder

1992 • 125 citations

Isolation of peroxisome assembly mutants from Saccharomyces cerevisiae with different morphologies using a novel positive selection procedure.

1992 • 123 citations

Differential protein import deficiencies in human peroxisome assembly disorders.

1994 • 117 citations

Human alkyldihydroxyacetonephosphate synthase deficiency: A new peroxisomal disorder

1994 • 115 citations

Peroxisome assembly factor–2, a putative ATPase cloned by functional complementation on a peroxisome–deficient mammalian cell mutant

1995 • 113 citations

Peb1p (Pas7p) is an intraperoxisomal receptor for the NH2-terminal, type 2, peroxisomal targeting sequence of thiolase: Peb1p itself is targeted to peroxisomes by an NH2-terminal peptide.

1996 • 100 citations

Intracellular protein trafficking defects in human disease

1992 • 96 citations

Proteins involved in peroxisome biogenesis and functioning

1996 • 83 citations

Centromeric DNA of Kluyveromyces lactis

1990 • 68 citations