Prominent publications by Joseph L Goldstein

KOL Index score: 17547

Sterol regulatory element binding protein-1 (SREBP1) and SREBP2 are structurally related proteins that control cholesterol homeostasis by stimulating transcription of sterol-regulated genes, including those encoding the low-density lipoprotein (LDL) receptor and 3-hydroxy-3-methylglutaryl CoA synthase. SREBP1 and SREBP2 are 47% identical, and they share a novel structure comprising a transcriptionally active NH2-terminal basic helix-loop-helix-leucine zipper (bHLH-Zip) domain followed by ...

Known for Element Binding |  Sterol Regulatory |  Human Pair |  Srebp1 Srebp2 |  Alternative Splicing
KOL Index score: 17491

The low density lipoprotein receptor-related protein/alpha 2-macroglobulin receptor (LRP) and gp330, two members of the low density lipoprotein receptor gene family, share a multitude of cysteine-rich repeats. LRP has been shown to act as an endocytosis-mediating receptor for several ligands, including protease-antiprotease complexes and plasma lipoproteins. The former include alpha 2-macroglobulin-protease complexes and plasminogen activator inhibitor-activator complexes. The latter ...

Known for Lrp Gp330 |  Lipoprotein Receptor |  Low Density |  Plasminogen Activator |  Inhibitor Complexes
KOL Index score: 17395

Animals fed cholesterol accumulate several types of cholesterol-rich lipoproteins in their plasma and ultimately develop cholesteryl ester deposition in tissue macrophages. Previous studies in the cholesterol-fed dog have shown that one class of cholesterol-rich lipoproteins. beta-migrating very low density lipoproteins (beta-VLDL, density < 1.006 g/ml), possesses a unique ability to produce cellular cholesteryl ester accumulation when incubated with mouse peritoneal macrophages in ...

Known for Cholesteryl Ester Synthesis |  Fed Animals |  Density Lipoproteins |  Vldl Cholesterol |  Accumulation Macrophages
KOL Index score: 17272

Rabbit livers express two genetically distinct receptors for plasma lipoproteins: (i) the low density lipoprotein (LDL) receptor and (ii) the chylomicron remnant receptor. In homozygous Watanabe-heritable hyperlipidemic (WHHL) rabbits, an animal model for human familial hypercholesterolemia, LDL receptors are genetically deficient, but chylomicron remnant receptors are normal. Hence, WHHL rabbits clear LDL from the circulation at an abnormally slow rate, but they clear chylomicron ...

Known for Whhl Rabbits |  Low Density |  Ldl Lipoproteins |  Animal Fasting |  125i Labeled
KOL Index score: 17154

Lipoproteins in blood plasma have been quantified and characterized in homozygous Watanabe-heritable hyperlipidemic (WHHL) rabbits, an animal model of human familial hypercholesterolemia. Like homozygous human hypercholesterolemics, WHHL rabbits have a severe deficiency of low density lipoprotein (LDL) receptors, a prolonged residence time for LDL, and an increased absolute rate of LDL catabolism. Although lipoproteins containing apolipoprotein B in WHHL rabbits are enriched in ...

Known for Animal Model |  Blood Plasma |  Whhl Rabbits |  Ldl Lipoproteins |  Hdl Cholesterol
KOL Index score: 16993

The activity of 3-hydroxy-3-methylglutaryl coenzyme A reductase, the rate-controlling enzyme in cholesterol biosynthesis, is suppressed in normal fibroblasts cultured in medium containing whole serum. The factor responsible for this suppression was localized to the low density and very low density lipoproteins, but was not found in high density lipoproteins. Whole serum from a patient with abetalipoproteinemia, which is deficient in apolipoprotein B (the protein common to low density and ...

Known for Methylglutaryl Coenzyme |  Reductase Activity |  Normal Subject |  Hdl Lipoproteins |  Low Density
KOL Index score: 16522

The current paper demonstrates that cholesterol and its hydroxylated derivative, 25-hydroxycholesterol (25-HC), inhibit cholesterol synthesis by two different mechanisms, both involving the proteins that control sterol regulatory element-binding proteins (SREBPs), membrane-bound transcription factors that activate genes encoding enzymes of lipid synthesis. Using methyl-beta-cyclodextrin as a delivery vehicle, we show that cholesterol enters cultured Chinese hamster ovary cells and ...

Known for Cholesterol Scap |  Binding Proteins |  25 Hc |  Regulatory Element |  Lipid Synthesis
KOL Index score: 16421

These studies were undertaken to measure rates of synthesis of digitonin-precipitable sterols in vivo and in vitro in control rabbits (New Zealand (NZ) control) and in homozygous Watanabe heritable hyperlipidemic rabbits (WHHL) that lack receptors for low density lipoproteins (LDL). The plasma cholesterol concentration in NZ control fetuses equaled 79 mg/dl, rose to 315 mg/dl 12 days after birth, and fell to 80 mg/dl in young adult animals. At these same ages, cholesterol concentrations ...

Known for Cholesterol Synthesis |  Whhl Rabbit |  Low Density |  Ldl Liver |  Adrenal Gland
KOL Index score: 16348

The proteolytic degradation of 125I-labeled low density lipoprotein by monolayers of cultured human fibroblasts was prevented by exposure of the cells to chloroquine, an agent that has been reported previously to inhibit lysosomal degradative processes. Chloroquine did not inhibit the binding of low density lipoprotein to its cell surface receptor. However, the two regulatory actions that normally follow low density lipoprotein binding to its receptor, namely suppression of ...

Known for Low Density Lipoprotein |  Proteolytic Degradation |  Triton Wr |  Reductase Activity |  Chloroquine Concanavalin
KOL Index score: 16143

In cultured human fibroblasts, each LDL receptor mediates the internalization of approximately 100 particles of LDL every 20 hr. We provide evidence that this reutilization of LDL receptors involves the recycling of receptors into and out of the cell and that the carboxylic ionophore monensin blocks the return of the receptors to the surface. In the presence of monensin and LDL, 75% of the receptors disappeared from the cell surface within 15 min and more than 90% disappeared within 60 ...

Known for Ldl Monensin |  Receptors Cell Surface |  Human Fibroblasts |  Density Lipoprotein |  Cell Membrane
KOL Index score: 16128

125I-Labeled low density lipoproteins were found to associate with monolayers of cultured normal fibroblasts by two processes—one of high affinity and one of low affinity. The high affinity association appeared to represent binding of the low density lipoprotein to specific receptor sites on the cell surface. This binding process exhibited saturation kinetics at low concentrations of the lipoprotein and competition by related molecules such as very low density lipoproteins. In addition, ...

Known for Low Density Lipoproteins |  Familial Hypercholesterolemia |  Normal Subject |  Culture Medium |  Affinity Binding
KOL Index score: 16097

We have produced transgenic mice whose livers express a dominant positive NH2-terminal fragment of sterol regulatory element binding protein-1c (SREBP-1c). Unlike full-length SREBP-1c, the NH2-terminal fragment enters the nucleus without a requirement for proteolytic release from cell membranes, and hence it is immune to downregulation by sterols. We compared SREBP-1c transgenic mice with a line of transgenic mice that produces an equal amount of the NH2-terminal fragment of SREBP-1a. ...

Known for Transgenic Mice |  Cultured Cells |  Messenger Receptors |  Sterol Regulatory Element |  Binding Protein
KOL Index score: 16092

Sterol regulatory element binding proteins (SREBP-1 and SREBP-2) are attached to the endoplasmic reticulum (ER) and nuclear envelope by a hairpin domain consisting of two transmembrane regions connected by a short lumenal loop of approximately 30 hydrophilic amino acids. In sterol-depleted cells, a protease cleaves the protein in the region of the first transmembrane domain, releasing an NH2-terminal fragment of approximately 500 amino acids that activates transcription of genes encoding ...

Known for Binding Proteins |  Endoplasmic Reticulum |  Regulatory Element |  Regulated Cleavage |  Transmembrane Domain
KOL Index score: 16050

Cellular cholesterol homeostasis is controlled by sterol-regulated proteolysis of membrane-bound transcription factors called sterol-regulatory element binding proteins (SREBPs). CPP32, a cysteine protease, was shown previously to cleave SREBP-1 and SREBP-2 in vitro at an aspartic acid between the basic helix-loop-helix leucine zipper domain and the first trans-membrane domain, liberating a transcriptionally active fragment. Here, we show that CPP32 exists in an inactive 32 kDa form in ...

Known for Binding Proteins |  Regulatory Element |  Cpp32 Apoptosis |  Cysteine Protease |  Cleavage Sterol
KOL Index score: 16028

Hepatic catabolism of lipoproteins containing apolipoproteins B or E is enhanced in rats treated with pharmacologic doses of 17 alpha-ethinyl estradiol. Liver membranes prepared from these rats exhibit an increased number of receptor sites that bind 125I-labeled human low density lipoproteins (LDL) in vitro. In the present studies, this estradiol-stimulated hepatic receptor was shown to recognize the following rat lipoproteins: LDL, very low density lipoproteins obtained from liver ...

Known for Rat Liver |  Binding Site |  Ldl Lipoproteins |  Lipoprotein Receptor |  Estradiol Humans


Joseph L Goldstein: Influence Statistics

Sample of concepts for which Joseph L Goldstein is among the top experts in the world.
Concept World rank
cholesterol synthesis lipoprotein #1
shortchain aldehydes enzyme #1
combinations cysteinerich repeats #1
cells exogenous cholesterol #1
membranespanning region #1
physiological 20th century #1
sterolmediated repression #1
53kda protein cells #1
membrane copii binding #1
sterols enzyme #1
lipoproteins igg15c8 #1
intertwined luminal loops #1
srebps endoplasmic reticulum #1
cholesterol transcription factors #1
complex coohterminal domain #1
proline202 #1
acs cytosolic enzyme #1
rhob ftase #1
synthesis 53k protein #1
cholesterol srebps #1
sterolregulated escort protein #1
cyscys geranylgeranylated proteins #1
srebp2 s1p #1
testicular feminization binding #1
srebp introns #1
sterolmediated regulation #1
coated vesicles fibroblasts #1
transcription presence #1
sterolregulated cleavage #1
cholesterol overaccumulation #1
sre1 absence #1
cys rab #1
direct srebp #1
normal rabbits mevinolin #1
clearance ldl receptor #1
whhl fibroblasts receptor #1
ldl receptor cdna #1
farnesyltransferase geranylgeranyltransferase #1
repression 25hydroxycholesterol #1
epiinsigdko mice #1
hamster liver srebp2 #1
hras farnesylation #1
helices immunoprecipitation #1
heterozygotes familial hypercholesterolemia #1
bza5b current studies #1
insigs reticulum farnesol #1
constitutively expressed cdna #1
farnesylated protein pxf #1
cholesterol synthesis srebps #1
familial hypercholesterolemia suppression #1

Key People For Ldl Receptor

Top KOLs in the world
Joseph L Goldstein
ldl receptor low density lipoprotein familial hypercholesterolemia
Michael S Brown
ldl receptor low density lipoprotein familial hypercholesterolemia
Joachim Joachim
lipoprotein receptors knockout mice ldl receptor
David Warwick Russell
ldl receptor bile acid 5 alpha
Wolfgang Johann Schneider
ldl receptor low density lipoprotein familial hypercholesterolemia
Helen H Hobbs
plasma levels transgenic mice african americans

Joseph L Goldstein:Expert Impact

Concepts for whichJoseph L Goldsteinhas direct influence:Ldl receptor,  Low density lipoprotein,  Familial hypercholesterolemia,  Ldl receptors,  Low density,  Density lipoprotein,  Human fibroblasts,  Binding proteins.

Joseph L Goldstein:KOL impact

Concepts related to the work of other authors for whichfor which Joseph L Goldstein has influence:Lipid metabolism,  Insulin resistance,  Gene expression,  Density lipoprotein,  Endoplasmic reticulum,  Ldl receptor,  Familial hypercholesterolemia.



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Department of Molecular Genetics, University of Texas Southwestern Medical Center, Dallas, TX 75390; | Department of Molecular Genetics, The University of Texas Southwestern Medical Cent

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