Genetic variation of human mononuclear leukocyte lysosomal acid lipase activity. Relationship to atherosclerosis.

PubWeight™: 0.85‹?›

🔗 View Article (PMID 3778571)

Published in Atherosclerosis on October 01, 1986

Authors

P M Coates, T Langer, J A Cortner

Articles by these authors

Chaperonin-mediated protein folding at the surface of groEL through a 'molten globule'-like intermediate. Nature (1991) 6.20

DnaK, DnaJ and GrpE form a cellular chaperone machinery capable of repairing heat-induced protein damage. EMBO J (1993) 4.32

DnaJ-like proteins: molecular chaperones and specific regulators of Hsp70. Trends Biochem Sci (1994) 3.65

The metabolism of low density lipoprotein in familial type II hyperlipoproteinemia. J Clin Invest (1972) 3.43

The ATP hydrolysis-dependent reaction cycle of the Escherichia coli Hsp70 system DnaK, DnaJ, and GrpE. Proc Natl Acad Sci U S A (1994) 3.33

The YTA10-12 complex, an AAA protease with chaperone-like activity in the inner membrane of mitochondria. Cell (1996) 2.80

A dual role for mitochondrial heat shock protein 70 in membrane translocation of preproteins. J Cell Biol (1993) 2.61

Molecular chaperones in protein folding: the art of avoiding sticky situations. Trends Biochem Sci (1994) 2.44

Prohibitins regulate membrane protein degradation by the m-AAA protease in mitochondria. Mol Cell Biol (1999) 2.34

The reaction cycle of GroEL and GroES in chaperonin-assisted protein folding. Nature (1993) 2.30

AAA proteases with catalytic sites on opposite membrane surfaces comprise a proteolytic system for the ATP-dependent degradation of inner membrane proteins in mitochondria. EMBO J (1996) 2.00

Medium-chain acyl-CoA dehydrogenase deficiency in children with non-ketotic hypoglycemia and low carnitine levels. Pediatr Res (1983) 1.91

Molecular lesion in patients with medium-chain acyl-CoA dehydrogenase deficiency. Lancet (1990) 1.82

Molecular chaperones cooperate with PIM1 protease in the degradation of misfolded proteins in mitochondria. EMBO J (1994) 1.80

Genetic deficiency of medium-chain acyl coenzyme A dehydrogenase: studies in cultured skin fibroblasts and peripheral mononuclear leukocytes. Pediatr Res (1985) 1.75

Membrane protein degradation by AAA proteases in mitochondria: extraction of substrates from either membrane surface. Mol Cell (2000) 1.73

Control of folding and membrane translocation by binding of the chaperone DnaJ to nascent polypeptides. Proc Natl Acad Sci U S A (1993) 1.72

Chaperone-like activity of the AAA domain of the yeast Yme1 AAA protease. Nature (1999) 1.62

Long-chain acyl coenzyme A dehydrogenase deficiency: an inherited cause of nonketotic hypoglycemia. Pediatr Res (1985) 1.56

The formation of respiratory chain complexes in mitochondria is under the proteolytic control of the m-AAA protease. EMBO J (1998) 1.56

Why drugs fail--a study on side effects in new chemical entities. Curr Pharm Des (2005) 1.54

Role of the ABC transporter Mdl1 in peptide export from mitochondria. Science (2001) 1.53

Molecular basis of medium chain acyl-coenzyme A dehydrogenase deficiency. An A to G transition at position 985 that causes a lysine-304 to glutamate substitution in the mature protein is the single prevalent mutation. J Clin Invest (1990) 1.51

Medium-chain acyl-CoA dehydrogenase deficiency. Diagnosis by stable-isotope dilution measurement of urinary n-hexanoylglycine and 3-phenylpropionylglycine. N Engl J Med (1988) 1.49

Kidney instant monitoring (K.IN.G): a new analyzer to monitor kidney function. Minerva Anestesiol (2010) 1.47

Yta10p is required for the ATP-dependent degradation of polypeptides in the inner membrane of mitochondria. FEBS Lett (1994) 1.46

Identification of very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase deficiency. Pediatr Res (1993) 1.46

Thyroid function in paediatric and young adult patients after sarcoma therapy: a report from the Late Effects Surveillance System. Clin Endocrinol (Oxf) (2007) 1.42

Medium-chain acyl-CoA dehydrogenase deficiency: postmortem diagnosis in a case of sudden infant death and neonatal diagnosis of an affected sibling. Pediatr Pathol (1992) 1.41

Age-related changes in cardiovascular disease risk factors of hypercholesterolemic children. J Pediatr (1998) 1.40

Acute muscular syndrome associated with administration of clofibrate. N Engl J Med (1968) 1.40

Molecular survey of a prevalent mutation, 985A-to-G transition, and identification of five infrequent mutations in the medium-chain Acyl-CoA dehydrogenase (MCAD) gene in 55 patients with MCAD deficiency. Am J Hum Genet (1991) 1.38

Hsp78, a Clp homologue within mitochondria, can substitute for chaperone functions of mt-hsp70. EMBO J (1995) 1.36

Chronic cardiomyopathy and weakness or acute coma in children with a defect in carnitine uptake. Ann Neurol (1991) 1.33

Biosynthesis of variant medium chain acyl-CoA dehydrogenase in cultured fibroblasts from patients with medium chain acyl-CoA dehydrogenase deficiency. Pediatr Res (1986) 1.28

Identification of a common mutation in patients with medium-chain acyl-CoA dehydrogenase deficiency. Biochem Biophys Res Commun (1990) 1.27

Afferents to the flocculus of the cerebellum in the rhesus macaque as revealed by retrograde transport of horseradish peroxidase. J Comp Neurol (1985) 1.26

Genetic variant of human erythrocyte malate dehydrogenase. Nature (1967) 1.24

Mitochondrial Hsp78, a member of the Clp/Hsp100 family in Saccharomyces cerevisiae, cooperates with Hsp70 in protein refolding. FEBS Lett (2001) 1.23

Plasma exchange in myasthenia gravis. Lancet (1977) 1.23

Genetic polymorphisms of human mitochondrial glutamic oxaloacetic transaminase. Science (1970) 1.21

Yta10p, a member of a novel ATPase family in yeast, is essential for mitochondrial function. FEBS Lett (1994) 1.20

Primary carnitine deficiency due to a failure of carnitine transport in kidney, muscle, and fibroblasts. N Engl J Med (1988) 1.19

Genetic variation of lysosomal acid lipase. Pediatr Res (1976) 1.19

Relationship between unusual hepatic acyl coenzyme A profiles and the pathogenesis of Reye syndrome. J Clin Invest (1988) 1.16

CNS late effects after ALL therapy in childhood. Part I: Neuroradiological findings in long-term survivors of childhood ALL--an evaluation of the interferences between morphology and neuropsychological performance. The German Late Effects Working Group. Med Pediatr Oncol (1997) 1.14

Medium-chain and long-chain acyl CoA dehydrogenase deficiency: clinical, pathologic and ultrastructural differentiation from Reye's syndrome. Hepatology (1987) 1.11

Mitochondrial malate dehydrogenase: a new genetic polymorphism in man. Science (1967) 1.10

Mutations in the medium chain acyl-CoA dehydrogenase (MCAD) gene. Hum Mutat (1992) 1.07

Afferents to the abducens nucleus in the monkey and cat. J Comp Neurol (1986) 1.07

Reduction of elevated LDL-cholesterol levels of 4- to 10-year-old children through home-based dietary education. Pediatrics (1994) 1.06

Use of metabolically competent human hepatoma cells for the detection of mutagens and antimutagens. Mutat Res (1998) 1.06

Conserved Ca2+-antagonist-binding properties and putative folding structure of a recombinant high-affinity dihydropyridine-binding domain. Biochem J (2000) 1.06

ATP-dependent proteolysis in mitochondria. m-AAA protease and PIM1 protease exert overlapping substrate specificities and cooperate with the mtHsp70 system. J Biol Chem (1998) 1.05

Mutations at the lysosomal acid cholesteryl ester hydrolase gene locus in Wolman disease. Proc Natl Acad Sci U S A (1994) 1.04

Direct measurement of apolipoprotein B synthesis in human very low density lipoprotein using stable isotopes and mass spectrometry. J Lipid Res (1986) 1.04

Strategies for efficient lead structure discovery from natural products. Curr Med Chem (2006) 1.04

Ifosfamide-induced nephrotoxicity in 593 sarcoma patients: a report from the Late Effects Surveillance System. Pediatr Blood Cancer (2007) 1.03

Early diagnosis and treatment of neonatal medium-chain acyl-CoA dehydrogenase deficiency: report of two siblings. Eur J Pediatr (1990) 1.01

Role of the mitochondrial DnaJ homologue, Mdj1p, in the prevention of heat-induced protein aggregation. FEBS Lett (1996) 1.00

Structural requirements of Tom40 for assembly into preexisting TOM complexes of mitochondria. Mol Biol Cell (2001) 0.99

Substitution of PIM1 protease in mitochondria by Escherichia coli Lon protease. J Biol Chem (1996) 0.99

Mapping and fitting the peripheral benzodiazepine receptor binding site by carboxamide derivatives. Comparison of different approaches to quantitative ligand-receptor interaction modeling. J Med Chem (2001) 0.98

Immunochemical characterization of variant long-chain acyl-CoA dehydrogenase in cultured fibroblasts from nine patients with long-chain acyl-CoA dehydrogenase deficiency. Pediatr Res (1991) 0.98

Protein degradation in mitochondria. Semin Cell Dev Biol (2000) 0.97

Plasmon electron-hole resonance in epitaxial graphene. J Phys Condens Matter (2010) 0.97

The chaperonin-related protein Tcm62p ensures mitochondrial gene expression under heat stress. FEBS Lett (2000) 0.97

Acid lipase activity of human lymphocytes. Biochim Biophys Acta (1979) 0.96

Floccular efferents in the rhesus macaque as revealed by autoradiography and horseradish peroxidase. J Comp Neurol (1985) 0.96

Familial combined hyperlipidemia in children: clinical expression, metabolic defects, and management. J Pediatr (1993) 0.96

The ATP-dependent PIM1 protease is required for the expression of intron-containing genes in mitochondria. Genes Dev (1998) 0.96

Kinetics of chylomicron remnant clearance in normal and in hyperlipoproteinemic subjects. J Lipid Res (1987) 0.95

A preliminary genetic interpretation of the esterase isozymes of human tissues. Ann Hum Genet (1975) 0.95

Cisplatin-induced ototoxicity in osteosarcoma patients: a report from the late effects surveillance system. Cancer Invest (2005) 0.93

The molecular chaperone Hsp78 confers compartment-specific thermotolerance to mitochondria. J Cell Biol (1996) 0.92

ATP-dependent proteases controlling mitochondrial function in the yeast Saccharomyces cerevisiae. Cell Mol Life Sci (1999) 0.92

Prospective longitudinal evaluation of doxorubicin-induced cardiomyopathy in sarcoma patients: a report of the late effects surveillance system (LESS). Pediatr Blood Cancer (2006) 0.92

Enhancing drug discovery through in silico screening: strategies to increase true positives retrieval rates. Curr Med Chem (2008) 0.92

Effect of early nutrition on serum cholesterol levels in adult rats challenged with high fat diet. J Nutr (1983) 0.91

The L-3-hydroxyacyl-CoA dehydrogenase deficiency. Prog Clin Biol Res (1990) 0.90

Genetic variation in human erythrocyte acetylcholinesterase. Science (1972) 0.89

Prenatal diagnosis of medium-chain acyl-CoA dehydrogenase deficiency in family with sudden infant death. Lancet (1987) 0.89

Hypolipidemic drugs and lipoprotein metabolism. Adv Exp Med Biol (1972) 0.88

Separation and further characterization of human adipose tissue neutral and alkaline lipolytic activities. J Biol Chem (1967) 0.88

Autocatalytic processing of the ATP-dependent PIM1 protease: crucial function of a pro-region for sorting to mitochondria. EMBO J (1997) 0.88

Mcx1p, a ClpX homologue in mitochondria of Saccharomyces cerevisiae. FEBS Lett (1998) 0.88

MAP-1 and IAP-1, two novel AAA proteases with catalytic sites on opposite membrane surfaces in mitochondrial inner membrane of Neurospora crassa. Mol Biol Cell (2001) 0.87

Low density lipoprotein metabolism and lipoprotein cholesterol content in southwestern American Indians. J Lipid Res (1979) 0.86

Molecular correlates of the action of bis(ethyl)polyamines in breast cancer cell growth inhibition and apoptosis. Biochem Cell Biol (2000) 0.85

Neonatal presentation of I-cell disease. J Pediatr (1978) 0.84

Familial combined hyperlipidaemia: use of stable isotopes to demonstrate overproduction of very low-density lipoprotein apolipoprotein B by the liver. J Inherit Metab Dis (1991) 0.84

Application of the in combo screening approach for the discovery of non-alkaloid acetylcholinesterase inhibitors from Cichorium intybus. Curr Drug Discov Technol (2005) 0.84

Azinyl and diazinyl hydrazones derived from aryl N-heteroaryl ketones: synthesis and antiproliferative activity. J Med Chem (1997) 0.84

Multicentre analysis of anthracycline-induced cardiotoxicity in children following treatment according to the nephroblastoma studies SIOP No.9/GPOH and SIOP 93-01/GPOH. Med Pediatr Oncol (2002) 0.84