Decreasing hydraulic conductivity of Bruch's membrane: Relevance to photoreceptor survival and lipofuscinoses

Domain structure of mitochondrial and chloroplast targeting peptides

1989 • 1,191 citations

Mitochondrial protein import

1989 • 820 citations

Requirement for hsp70 in the mitochondrial matrix for translocation and folding of precursor proteins

1990 • 756 citations

Age-related Macular Degeneration Histopathologic Studies

1993 • 747 citations

Aging human RPE: morphometric analysis of macular, equatorial, and peripheral cells.

1984 • 556 citations

Aging changes in Bruch's membrane. A histochemical and morphologic study.

1990 • 404 citations

Mitochondrial Hsp70/MIM44 complex facilitates protein import

1994 • 391 citations

The topography and age relationship of lipofuscin concentration in the retinal pigment epithelium.

1978 • 384 citations

Mitochondrial ATP synthase subunit c storage in the ceroid‐lipofuscinoses (Batten disease)

1992 • 356 citations

Transport into mitochondria and intramitochondrial sorting of the Fe/S protein of ubiquinol-cytochrome c reductase

1986 • 340 citations

Cleavage-site motifs in mitochondrial targeting peptides

1990 • 326 citations

THE MITOCHONDRIAL PROTEIN IMPORT APPARATUS

1990 • 311 citations

Survey of amino-terminal proteolytic cleavage sites in mitochondrial precursor proteins: leader peptides cleaved by two matrix proteases share a three-amino acid motif.

1989 • 303 citations

Aging Changes in Bruch's Membrane

1990 • 294 citations

The Role of Hsp70 in Conferring Unidirectionality on Protein Translocation into Mitochondria

1994 • 269 citations

Analysis of Lipid Deposits Extracted From Human Macular and Peripheral Bruch's Membrane

1994 • 254 citations

Mitochondrial molecular chaperones: their role in protein translocation

1994 • 203 citations

Targeting of pre-ornithine transcarbamylase to mitochondria: Definition of critical regions and residues in the leader peptide

1986 • 172 citations

A study of the prevalence of eye disease in the elderly in an English community.

1985 • 153 citations

Cleavage of precursors by the mitochondrial processing peptidase requires a compatible mature protein or an intermediate octapeptide.

1991 • 130 citations

Scanning detection of mutations in human ornithine transcarbamoylase by chemical mismatch cleavage.

1989 • 129 citations

The protein import machine of the mitochondrial inner membrane

1994 • 121 citations

Rat liver mitochondrial intermediate peptidase (MIP): purification and initial characterization.

1992 • 107 citations

Mitochondrial protein import: Specific recognition and membrane translocation of preproteins

1993 • 102 citations

The Fine Structure of the Retina in Neuronal Ceroid-Lipofuscinosis

1974 • 85 citations

Import of rat ornithine transcarbamylase precursor into mitochondria: two-step processing of the leader peptide.

1987 • 76 citations

Arginine in the leader peptide is required for both import and proteolytic cleavage of a mitochondrial precursor.

1985 • 74 citations

Amino-terminal octapeptides function as recognition signals for the mitochondrial intermediate peptidase.

1992 • 67 citations

Mutation eliminating mitochondrial leader sequence of methylmalonyl-CoA mutase causes muto methylmalonic acidemia.

1990 • 49 citations

The ornithine transcarbamylase leader peptide directs mitochondrial import through both its midportion structure and net positive charge.

1987 • 44 citations

Influence of dietary vitamin a on autofluorescence of leupeptin-induced inclusions in the retinal pigment epithelium

1992 • 42 citations

Point Mutation Affecting Processing of the Ornithine Aminotransferase Precursor Protein in Gyrate Atrophy

1989 • 41 citations

Immunochemical studies of fibroblasts from patients with methylmalonyl-CoA mutase apoenzyme deficiency: detection of a mutation interfering with mitochondrial import.

1987 • 34 citations

A single amino acid substitution within the mature sequence of ornithine aminotransferase obstructs mitochondrial entry of the precursor.

1995 • 27 citations

Mitochondrial import and processing of mutant human ornithine transcarbamylase precursors in cultured cells.

1988 • 26 citations

Dietary restriction slows age pigment accumulation in the retinal pigment epithelium.

1993 • 25 citations

Different structures in the amino-terminal domain of the ornithine transcarbamylase leader peptide are involved in mitochondrial import and carboxyl-terminal cleavage.

1988 • 25 citations

Altered amyloid β-protein precursor processing in brains of patients with neuronal ceroid lipofuscinosis

1990 • 25 citations

The variant human isovaleryl-CoA dehydrogenase gene responsible for type II isovaleric acidemia determines an RNA splicing error, leading to the deletion of the entire second coding exon and the production of a truncated precursor protein that interacts poorly with mitochondrial import receptors.

1992 • 23 citations

Reduced phagosomal content of the retinal pigment epithelium in response to retinoid deprivation.

1992 • 22 citations

Stored dolichyl pyrophosphoryl oligosaccharides in Batten disease

1992 • 16 citations

Ceroid Lipofuscinosis

1975 • 16 citations

Decreased Erythrocyte and Platelet Phospholipids and Fatty Acids in Juvenile Neuronal Ceroid-Lipofuscinosis (Batten Disease)

1990 • 15 citations

Mitochondrial Import and Processing of Mutant Human Ornithine Transcarbamylase Precursors in Cultured Cells

1988 • 11 citations

Phospholipases and the Molecular Basis for the Formation of Ceroid in Batten Disease

1990 • 11 citations

Mitochondrial protein import

1990 • 2 citations