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

Molecular Cloning: A Laboratory Manual

2001 • 133,517 citations

Molecular cloning: A laboratory manual

1990 • 85,659 citations

DNA sequencing with chain-terminating inhibitors

1977 • 69,181 citations

Rapid production of full-length cDNAs from rare transcripts: amplification using a single gene-specific oligonucleotide primer.

1988 • 4,669 citations

The Metabolic Basis of Inherited Disease

1990 • 4,327 citations

A conserved tripeptide sorts proteins to peroxisomes.

1989 • 1,113 citations

Biogenesis of Peroxisomes

1985 • 1,108 citations

Biochemistry of dystrophic muscle. Mitochondrial succinate-tetrazolium reductase and adenosine triphosphatase

1961 • 1,041 citations

BIOCHEMISTRY OF PEROXISOMES

1992 • 843 citations

Translocation of proteins across the endoplasmic reticulum III. Signal recognition protein (SRP) causes signal sequence-dependent and site-specific arrest of chain elongation that is released by microsomal membranes.

1981 • 807 citations

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

1991 • 629 citations

Identification of a peroxisomal targeting signal at the carboxy terminus of firefly luciferase.

1987 • 481 citations

The TPR snap helix: a novel protein repeat motif from mitosis to transcription

1991 • 452 citations

Protein Import into Peroxisomes and Biogenesis of the Organelle

1993 • 411 citations

LYSOSOMES OF THE ARTERIAL WALL

1972 • 410 citations

Rapid cDNA sequencing (expressed sequence tags) from a directionally cloned human infant brain cDNA library

1993 • 361 citations

A Human Gene Responsible for Zellweger Syndrome That Affects Peroxisome Assembly

1992 • 357 citations

Peroxisomal Membrane Ghosts in Zellweger Syndrome—Aberrant Organelle Assembly

1988 • 327 citations

Isolation of peroxisome-deficient mutants of Saccharomyces cerevisiae.

1989 • 326 citations

Snap helix with knob and hole: Essential repeats in S. pombe nuclear protein nuc2 +

1990 • 306 citations

A mitochondrial import receptor for the ADP/ATP carrier

1990 • 296 citations

Peroxisomal protein import is conserved between yeast, plants, insects and mammals.

1990 • 296 citations

Disorders of peroxisome biogenesis

1995 • 289 citations

Amino-terminal presequence of the precursor of peroxisomal 3-ketoacyl-CoA thiolase is a cleavable signal peptide for peroxisomal targeting

1991 • 288 citations

Protein import into yeast mitochondria is accelerated by the outer membrane protein MAS70.

1990 • 276 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

Mutations in the 70K peroxisomal membrane protein gene in Zellweger syndrome

1992 • 228 citations

Functional cooperation of mitochondrial protein import receptors in yeast.

1993 • 227 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

Development of the yeast Pichia pastoris as a model organism for a genetic and molecular analysis of peroxisome assembly

1992 • 211 citations

Adrenoleukodystrophy: Elevated C26 fatty acid in cultured skin fibroblasts

1980 • 199 citations

Peroxisome Targeting Signal of Rat Liver Acyl-Coenzyme A Oxidase Resides at the Carboxy Terminus

1989 • 197 citations

Translocation of acyl-CoA oxidase into peroxisomes requires ATP hydrolysis but not a membrane potential.

1987 • 191 citations

Translocation of proteins across the endoplasmic reticulum

1993 • 176 citations

Evolutionary conservation of a microbody targeting signal that targets proteins to peroxisomes, glyoxysomes, and glycosomes.

1991 • 166 citations

Genetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions. A study using complementation analysis.

1988 • 164 citations

Mutagenesis of the amino targeting signal of Saccharomyces cerevisiae 3-ketoacyl-CoA thiolase reveals conserved amino acids required for import into peroxisomes in vivo.

1994 • 160 citations

Mutational analysis of the N-terminal topogenic signal of watermelon glyoxysomal malate dehydrogenase using the heterologous host Hansenula polymorpha.

1994 • 149 citations

Structural comparison of the Pichia pastoris alcohol oxidase genes

1989 • 143 citations

Involvement of 70-kD heat-shock proteins in peroxisomal import.

1994 • 134 citations

Antibodies directed against the peroxisomal targeting signal of firefly luciferase recognize multiple mammalian peroxisomal proteins.

1990 • 133 citations

Targeting efficiencies of various permutations of the consensus C‐terminal tripeptide peroxisomal targeting signal

1992 • 130 citations

Cytosol-dependent peroxisomal protein import in a permeabilized cell system.

1993 • 129 citations

Deletion of the receptor MOM19 strongly impairs import of cleavable preproteins into Saccharomyces cerevisiae mitochondria.

1994 • 124 citations

Identification of three distinct peroxisomal protein import defects in patients with peroxisome biogenesis disorders

1995 • 120 citations

Carboxyl-terminal consensus Ser-Lys-Leu-related tripeptide of peroxisomal proteins functions in vitro as a minimal peroxisome-targeting signal.

1992 • 119 citations

Strand-separating conformational polymorphism analysis: Efficacy of detection of point mutations in the human ornithine δ-aminotransferase gene

1992 • 118 citations

Peroxisome‐deficient mutants of Hansenula polymorpha

1990 • 117 citations

Differential protein import deficiencies in human peroxisome assembly disorders.

1994 • 117 citations

Peroxisome targeting signal of rat liver acyl-coenzyme A oxidase resides at the carboxy terminus.

1989 • 117 citations

Proton ionophores prevent assembly of a peroxisomal protein

1987 • 114 citations

An efficient screen for peroxisome-deficient mutants of Pichia pastoris

1992 • 113 citations

The Pichia pastoris PAS4 gene encodes a ubiquitin-conjugating enzyme required for peroxisome assembly.

1994 • 111 citations

The carboxyl-terminal tripeptide Ala-Lys-Ile is essential for targeting Candida tropicalis trifunctional enzyme to yeast peroxisomes.

1991 • 97 citations

Genetic and Phenotypic Heterogeneity in Disorders of Peroxisome Biogenesis—A Complementation Study Involving Cell Lines from 19 Patients

1989 • 97 citations

An efficient positive selection procedure for the isolation of peroxisomal import and peroxisome assembly mutants of Saccharomyces cerevisiae.

1993 • 92 citations

Peroxisomal fatty acid β-oxidation in HepG2 cells

1991 • 83 citations

PAY4, a gene required for peroxisome assembly in the yeast Yarrowia lipolytica, encodes a novel member of a family of putative ATPases.

1994 • 69 citations

Targeting signal of the peroxisomal catalase in the methylotrophic yeastHansenula polymorpha

1992 • 62 citations

p62cdc23 of Saccharomyces cerevisiae: a nuclear tetratricopeptide repeat protein with two mutable domains.

1993 • 60 citations

Targeting sequences of the two major peroxisomal proteins in the methylotrophic yeast Hansenula polymorpha

1992 • 56 citations

Import of Firefly Luciferase into Mammalian Peroxisomes in Vivo Requires Nucleoside Triphosphates

1993 • 53 citations

Myopathy in an infant with a fatal peroxisomal disorder

1986 • 29 citations

The Pichia pastoris HIS4 gene: nucleotide sequence, creation of a non-reverting his4 deletion mutant, and development of HIS4-based replicating and integrating plasmids

1994 • 29 citations

Restoration of peroxisome biogenesis in a peroxisome‐deficient mammalian cell line by expression of either the 35 kDa or the 70 kDa peroxisomal membrane proteins

1994 • 16 citations