List of works - Loren G Fong

10 Genetic approaches to understanding the physiologic importance of the carboxyl methylation of isoprenylated proteins

scientific article published on January 2006

A disordered acidic domain in GPIHBP1 harboring a sulfated tyrosine regulates lipoprotein lipase

scientific article published on 13 June 2018

A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation.

scientific article

A mouse monoclonal antibody specific for mouse apoB48 and apoB100 produced by immunizing "apoB39-only" mice with mouse apoB48

scientific article published on 01 February 2006

A novel approach to tag and identify geranylgeranylated proteins

scientific article

A protein farnesyltransferase inhibitor ameliorates disease in a mouse model of progeria.

scientific article

Abnormal development of the cerebral cortex and cerebellum in the setting of lamin B2 deficiency.

scientific article

Abnormal patterns of lipoprotein lipase release into the plasma in GPIHBP1-deficient mice.

scientific article published on 8 October 2008

Absence of progeria-like disease phenotypes in knock-in mice expressing a non-farnesylated version of progerin

scientific article

Adipose subtype-selective recruitment of TLE3 or Prdm16 by PPARγ specifies lipid storage versus thermogenic gene programs

scientific article published on March 2013

An LPL-specific monoclonal antibody, 88B8, that abolishes the binding of LPL to GPIHBP1.

scientific article published on 5 August 2016

An absence of both lamin B1 and lamin B2 in keratinocytes has no effect on cell proliferation or the development of skin and hair.

scientific article

An absence of nuclear lamins in keratinocytes leads to ichthyosis, defective epidermal barrier function, and intrusion of nuclear membranes and endoplasmic reticulum into the nuclear chromatin

scientific article

An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria

scientific article

An upstream enhancer regulates Gpihbp1 expression in a tissue-specific manner

scientific article published on 31 December 2018

Apolipoprotein C-III inhibits triglyceride hydrolysis by GPIHBP1-bound LPL.

scientific article

Are B-type lamins essential in all mammalian cells?

scientific article published in November 2011

Assessing mechanisms of GPIHBP1 and lipoprotein lipase movement across endothelial cells.

scientific article

Assessing the efficacy of protein farnesyltransferase inhibitors in mouse models of progeria

scientific article

Assessing the role of the glycosylphosphatidylinositol-anchored high density lipoprotein-binding protein 1 (GPIHBP1) three-finger domain in binding lipoprotein lipase

scientific article

Binding preferences for GPIHBP1, a glycosylphosphatidylinositol-anchored protein of capillary endothelial cells

scientific article

Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation.

scientific article

Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes

scientific article

Caution! Analyze transcripts from conditional knockout alleles

scientific article published on 18 December 2008

Cholesterol intake modulates plasma triglyceride levels in glycosylphosphatidylinositol HDL-binding protein 1-deficient mice

scientific article

Chylomicronemia From GPIHBP1 Autoantibodies Successfully Treated With Rituximab: A Case Report

scientific article published on 11 August 2020

Chylomicronemia mutations yield new insights into interactions between lipoprotein lipase and GPIHBP1

scientific article

Chylomicronemia with a mutant GPIHBP1 (Q115P) that cannot bind lipoprotein lipase

scientific article

Chylomicronemia with low postheparin lipoprotein lipase levels in the setting of GPIHBP1 defects

scientific article

Compromised mitochondrial fatty acid synthesis in transgenic mice results in defective protein lipoylation and energy disequilibrium

scientific article (publication date: 2012)

Cultured macrophages transfer surplus cholesterol into adjacent cells in the absence of serum or high-density lipoproteins

scientific article published on 30 April 2020

Deficiencies in lamin B1 and lamin B2 cause neurodevelopmental defects and distinct nuclear shape abnormalities in neurons

scientific article published on 5 October 2011

Deletion of the basement membrane heparan sulfate proteoglycan type XVIII collagen causes hypertriglyceridemia in mice and humans

scientific article

Direct synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice

scientific article

Disrupting the LINC complex in smooth muscle cells reduces aortic disease in a mouse model of Hutchinson-Gilford progeria syndrome

scientific article published on 01 September 2018

Do lamin B1 and lamin B2 have redundant functions?

scientific article

Eliminating the synthesis of mature lamin A reduces disease phenotypes in mice carrying a Hutchinson-Gilford progeria syndrome allele.

scientific article

Farnesylation of lamin B1 is important for retention of nuclear chromatin during neuronal migration ⬇️

scientific article published on May 6, 2013

Fibroblasts lacking nuclear lamins do not have nuclear blebs or protrusions but nevertheless have frequent nuclear membrane ruptures

article published in the Proceedings of the National Academy of Sciences of the United States of America

GPIHBP1 and Plasma Triglyceride Metabolism

scientific article published on 14 May 2016

GPIHBP1 and lipolysis: an update

scientific article

GPIHBP1 autoantibodies in a patient with unexplained chylomicronemia.

scientific article published on 13 June 2017

GPIHBP1 expression in gliomas promotes utilization of lipoprotein-derived nutrients.

scientific article published on 6 June 2019

GPIHBP1 is responsible for the entry of lipoprotein lipase into capillaries

scientific article

GPIHBP1 missense mutations often cause multimerization of GPIHBP1 and thereby prevent lipoprotein lipase binding.

scientific article published on 11 November 2014

GPIHBP1, a GPI-anchored protein required for the lipolytic processing of triglyceride-rich lipoproteins

scientific article published on 14 October 2008

GPIHBP1, an endothelial cell transporter for lipoprotein lipase ⬇️

scientific article published on August 15, 2011

GPIHBP1: an endothelial cell molecule important for the lipolytic processing of chylomicrons

scientific article

Genetic analyses of the role of RCE1 in RAS membrane association and transformation.

scientific article published on January 2008

Genetic studies on the functional relevance of the protein prenyltransferases in skin keratinocytes.

scientific article

Glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1 plays a critical role in the lipolytic processing of chylomicrons

scientific article

HIV protease inhibitors block the zinc metalloproteinase ZMPSTE24 and lead to an accumulation of prelamin A in cells.

scholarly article

HIV-protease inhibitors block the enzymatic activity of purified Ste24p.

scientific article published on 17 July 2008

Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice

scientific journal article

High-resolution imaging and quantification of plasma membrane cholesterol by NanoSIMS.

scientific article published on 6 February 2017

High-resolution imaging of dietary lipids in cells and tissues by NanoSIMS analysis.

scientific article

Highly conserved cysteines within the Ly6 domain of GPIHBP1 are crucial for the binding of lipoprotein lipase.

scientific article published on 02 September 2009

IDOL stimulates clathrin-independent endocytosis and multivesicular body-mediated lysosomal degradation of the low-density lipoprotein receptor

scientific article

Impaired thermogenesis and sharp increases in plasma triglyceride levels in GPIHBP1-deficient mice during cold exposure

scientific article published on 15 February 2018

Increased progerin expression associated with unusual LMNA mutations causes severe progeroid syndromes.

scientific article

Inhibitors of protein geranylgeranyltransferase-I lead to prelamin A accumulation in cells by inhibiting ZMPSTE24

scientific article

LINCing lamin B2 to neuronal migration: growing evidence for cell-specific roles of B-type lamins.

scientific article

LMNA missense mutations causing familial partial lipodystrophy do not lead to an accumulation of prelamin A.

scientific article published in September 2016

Lamin B1 and lamin B2 are long-lived proteins with distinct functions in retinal development.

scientific article published on 13 April 2016

Laminopathies and the long strange trip from basic cell biology to therapy

scientific article

Lipin 2/3 phosphatidic acid phosphatases maintain phospholipid homeostasis to regulate chylomicron synthesis

scientific article published on 03 December 2018

Lipins, lipinopathies, and the modulation of cellular lipid storage and signaling

scientific article

Lowering plasma cholesterol by raising LDL receptors--revisited.

scientific article published on March 2012

Macrophages release plasma membrane-derived particles rich in accessible cholesterol

scientific article published on 20 August 2018

Modeling insertional mutagenesis using gene length and expression in murine embryonic stem cells

scientific article

Modulation of LMNA splicing as a strategy to treat prelamin A diseases.

scientific article published on 21 March 2016

Monoclonal antibodies that bind to the Ly6 domain of GPIHBP1 abolish the binding of LPL.

scientific article published on 15 November 2016

Mouse lipin-1 and lipin-2 cooperate to maintain glycerolipid homeostasis in liver and aging cerebellum.

scientific article

Mouse models of the laminopathies.

scientific article

Multimerization of glycosylphosphatidylinositol-anchored high density lipoprotein-binding protein 1 (GPIHBP1) and familial chylomicronemia from a serine-to-cysteine substitution in GPIHBP1 Ly6 domain

scientific article

Mutating a conserved cysteine in GPIHBP1 reduces amounts of GPIHBP1 in capillaries and abolishes LPL binding.

scientific article published on 5 May 2017

Mutation of conserved cysteines in the Ly6 domain of GPIHBP1 in familial chylomicronemia

scientific article

Mutations in lipoprotein lipase that block binding to the endothelial cell transporter GPIHBP1

scientific article

NanoSIMS Analysis of Intravascular Lipolysis and Lipid Movement across Capillaries and into Cardiomyocytes

NanoSIMS imaging reveals unexpected heterogeneity in nutrient uptake by brown adipocytes

scientific article published on 15 September 2018

NanoSIMS imaging: an approach for visualizing and quantifying lipids in cells and tissues

scientific article published on 28 October 2016

New Lmna knock-in mice provide a molecular mechanism for the 'segmental aging' in Hutchinson-Gilford progeria syndrome

scientific article

New wrinkles in lipoprotein lipase biology.

scientific article

Normal binding of lipoprotein lipase, chylomicrons, and apo-AV to GPIHBP1 containing a G56R amino acid substitution

scientific article published in December 2007

Nuclear envelope protein Lem2 is required for mouse development and regulates MAP and AKT kinases

scientific journal article

Nuclear lamins and neurobiology

scientific article

Nuclear lamins in the brain - new insights into function and regulation.

scientific article published on 14 October 2012

PCSK9 function and physiology.

scientific article published on July 2008

Palmoplantar Keratoderma in Slurp2-Deficient Mice

scientific article

Palmoplantar keratoderma along with neuromuscular and metabolic phenotypes in Slurp1-deficient mice

scientific article

Peroxidasin-mediated bromine enrichment of basement membranes

scientific article published on 22 June 2020

Prelamin A causes aberrant myonuclear arrangement and results in muscle fiber weakness

scientific article published on 04 October 2018

Prelamin A farnesylation and progeroid syndromes

scientific article

Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated.

scientific article published on October 2008

Protein farnesylation inhibitors cause donut-shaped cell nuclei attributable to a centrosome separation defect

scientific article

Protein farnesyltransferase inhibitors and progeria

scientific article

Reciprocal knock-in mice to investigate the functional redundancy of lamin B1 and lamin B2.

scientific article

Reciprocal metabolic perturbations in the adipose tissue and liver of GPIHBP1-deficient mice.

scientific article published on 15 December 2011

Regulation of prelamin A but not lamin C by miR-9, a brain-specific microRNA.

scientific article published on 30 January 2012

Release of cholesterol-rich particles from the macrophage plasma membrane during movement of filopodia and lamellipodia

scientific article published on 05 September 2019

SREBP-2-deficient and hypomorphic mice reveal roles for SREBP-2 in embryonic development and SREBP-1c expression

scientific article published on 18 December 2015

Severe hepatocellular disease in mice lacking one or both CaaX prenyltransferases.

scientific article published on 28 October 2011

Sphingosine-1-phosphate lyase downregulation promotes colon carcinogenesis through STAT3-activated microRNAs

scientific article

Sphingosine-1-phosphate lyase expression in embryonic and adult murine tissues

scientific article

Sterol regulatory element binding protein 1a regulates hepatic fatty acid partitioning by activating acetyl coenzyme A carboxylase 2

scientific journal article

Structure of the lipoprotein lipase-GPIHBP1 complex that mediates plasma triglyceride hydrolysis

scientific article published on 17 December 2018

Targeting isoprenylcysteine methylation ameliorates disease in a mouse model of progeria.

scientific article

Targeting protein prenylation in progeria

scientific article

The GPIHBP1-LPL complex is responsible for the margination of triglyceride-rich lipoproteins in capillaries

scientific article published on 10 April 2014

The acidic domain of the endothelial membrane protein GPIHBP1 stabilizes lipoprotein lipase activity by preventing unfolding of its catalytic domain.

scientific article published on 3 January 2016

The arrestin domain-containing 3 protein regulates body mass and energy expenditure

scientific article

The expression of GPIHBP1, an endothelial cell binding site for lipoprotein lipase and chylomicrons, is induced by peroxisome proliferator-activated receptor-gamma

scientific article published on 11 September 2008

The posttranslational processing of prelamin A and disease.

scientific article

Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson-Gilford progeria syndrome mutation.

scientific article

Unexpected expression pattern for glycosylphosphatidylinositol-anchored HDL-binding protein 1 (GPIHBP1) in mouse tissues revealed by positron emission tomography scanning.

scientific article

List of works by Loren G Fong

10 Genetic approaches to understanding the physiologic importance of the carboxyl methylation of isoprenylated proteins

A disordered acidic domain in GPIHBP1 harboring a sulfated tyrosine regulates lipoprotein lipase

A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation.

A mouse monoclonal antibody specific for mouse apoB48 and apoB100 produced by immunizing "apoB39-only" mice with mouse apoB48

A novel approach to tag and identify geranylgeranylated proteins

A protein farnesyltransferase inhibitor ameliorates disease in a mouse model of progeria.

Abnormal development of the cerebral cortex and cerebellum in the setting of lamin B2 deficiency.

Abnormal patterns of lipoprotein lipase release into the plasma in GPIHBP1-deficient mice.

Absence of progeria-like disease phenotypes in knock-in mice expressing a non-farnesylated version of progerin

Adipose subtype-selective recruitment of TLE3 or Prdm16 by PPARγ specifies lipid storage versus thermogenic gene programs

An LPL-specific monoclonal antibody, 88B8, that abolishes the binding of LPL to GPIHBP1.

An absence of both lamin B1 and lamin B2 in keratinocytes has no effect on cell proliferation or the development of skin and hair.

An absence of nuclear lamins in keratinocytes leads to ichthyosis, defective epidermal barrier function, and intrusion of nuclear membranes and endoplasmic reticulum into the nuclear chromatin

An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria

An upstream enhancer regulates Gpihbp1 expression in a tissue-specific manner

Apolipoprotein C-III inhibits triglyceride hydrolysis by GPIHBP1-bound LPL.

Are B-type lamins essential in all mammalian cells?

Assessing mechanisms of GPIHBP1 and lipoprotein lipase movement across endothelial cells.

Assessing the efficacy of protein farnesyltransferase inhibitors in mouse models of progeria

Assessing the role of the glycosylphosphatidylinositol-anchored high density lipoprotein-binding protein 1 (GPIHBP1) three-finger domain in binding lipoprotein lipase

Binding preferences for GPIHBP1, a glycosylphosphatidylinositol-anchored protein of capillary endothelial cells

Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation.

Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes

Caution! Analyze transcripts from conditional knockout alleles

Cholesterol intake modulates plasma triglyceride levels in glycosylphosphatidylinositol HDL-binding protein 1-deficient mice

Chylomicronemia From GPIHBP1 Autoantibodies Successfully Treated With Rituximab: A Case Report

Chylomicronemia mutations yield new insights into interactions between lipoprotein lipase and GPIHBP1

Chylomicronemia with a mutant GPIHBP1 (Q115P) that cannot bind lipoprotein lipase

Chylomicronemia with low postheparin lipoprotein lipase levels in the setting of GPIHBP1 defects

Compromised mitochondrial fatty acid synthesis in transgenic mice results in defective protein lipoylation and energy disequilibrium

Cultured macrophages transfer surplus cholesterol into adjacent cells in the absence of serum or high-density lipoproteins

Deficiencies in lamin B1 and lamin B2 cause neurodevelopmental defects and distinct nuclear shape abnormalities in neurons

Deletion of the basement membrane heparan sulfate proteoglycan type XVIII collagen causes hypertriglyceridemia in mice and humans

Direct synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice

Disrupting the LINC complex in smooth muscle cells reduces aortic disease in a mouse model of Hutchinson-Gilford progeria syndrome

Do lamin B1 and lamin B2 have redundant functions?

Eliminating the synthesis of mature lamin A reduces disease phenotypes in mice carrying a Hutchinson-Gilford progeria syndrome allele.

Farnesylation of lamin B1 is important for retention of nuclear chromatin during neuronal migration ⬇️

Fibroblasts lacking nuclear lamins do not have nuclear blebs or protrusions but nevertheless have frequent nuclear membrane ruptures

GPIHBP1 and Plasma Triglyceride Metabolism

GPIHBP1 and lipolysis: an update

GPIHBP1 autoantibodies in a patient with unexplained chylomicronemia.

GPIHBP1 expression in gliomas promotes utilization of lipoprotein-derived nutrients.

GPIHBP1 is responsible for the entry of lipoprotein lipase into capillaries

GPIHBP1 missense mutations often cause multimerization of GPIHBP1 and thereby prevent lipoprotein lipase binding.

GPIHBP1, a GPI-anchored protein required for the lipolytic processing of triglyceride-rich lipoproteins

GPIHBP1, an endothelial cell transporter for lipoprotein lipase ⬇️

GPIHBP1: an endothelial cell molecule important for the lipolytic processing of chylomicrons

Genetic analyses of the role of RCE1 in RAS membrane association and transformation.

Genetic studies on the functional relevance of the protein prenyltransferases in skin keratinocytes.

Glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1 plays a critical role in the lipolytic processing of chylomicrons

HIV protease inhibitors block the zinc metalloproteinase ZMPSTE24 and lead to an accumulation of prelamin A in cells.

HIV-protease inhibitors block the enzymatic activity of purified Ste24p.

Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice

High-resolution imaging and quantification of plasma membrane cholesterol by NanoSIMS.

High-resolution imaging of dietary lipids in cells and tissues by NanoSIMS analysis.

Highly conserved cysteines within the Ly6 domain of GPIHBP1 are crucial for the binding of lipoprotein lipase.

IDOL stimulates clathrin-independent endocytosis and multivesicular body-mediated lysosomal degradation of the low-density lipoprotein receptor

Impaired thermogenesis and sharp increases in plasma triglyceride levels in GPIHBP1-deficient mice during cold exposure

Increased progerin expression associated with unusual LMNA mutations causes severe progeroid syndromes.

Inhibitors of protein geranylgeranyltransferase-I lead to prelamin A accumulation in cells by inhibiting ZMPSTE24

LINCing lamin B2 to neuronal migration: growing evidence for cell-specific roles of B-type lamins.

LMNA missense mutations causing familial partial lipodystrophy do not lead to an accumulation of prelamin A.

Lamin B1 and lamin B2 are long-lived proteins with distinct functions in retinal development.

Laminopathies and the long strange trip from basic cell biology to therapy

Lipin 2/3 phosphatidic acid phosphatases maintain phospholipid homeostasis to regulate chylomicron synthesis

Lipins, lipinopathies, and the modulation of cellular lipid storage and signaling

Lowering plasma cholesterol by raising LDL receptors--revisited.

Macrophages release plasma membrane-derived particles rich in accessible cholesterol

Modeling insertional mutagenesis using gene length and expression in murine embryonic stem cells

Modulation of LMNA splicing as a strategy to treat prelamin A diseases.

Monoclonal antibodies that bind to the Ly6 domain of GPIHBP1 abolish the binding of LPL.

Mouse lipin-1 and lipin-2 cooperate to maintain glycerolipid homeostasis in liver and aging cerebellum.

Mouse models of the laminopathies.

Multimerization of glycosylphosphatidylinositol-anchored high density lipoprotein-binding protein 1 (GPIHBP1) and familial chylomicronemia from a serine-to-cysteine substitution in GPIHBP1 Ly6 domain

Mutating a conserved cysteine in GPIHBP1 reduces amounts of GPIHBP1 in capillaries and abolishes LPL binding.

Mutation of conserved cysteines in the Ly6 domain of GPIHBP1 in familial chylomicronemia

Mutations in lipoprotein lipase that block binding to the endothelial cell transporter GPIHBP1

NanoSIMS Analysis of Intravascular Lipolysis and Lipid Movement across Capillaries and into Cardiomyocytes