Richard S. Brush
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View article: Cinnabarinic acid protects against metabolic dysfunction-associated steatohepatitis by activating aryl hydrocarbon receptor-dependent AMPK signaling
Cinnabarinic acid protects against metabolic dysfunction-associated steatohepatitis by activating aryl hydrocarbon receptor-dependent AMPK signaling Open
The study showed that the absence of AhR in hepatocytes results in exacerbated metabolic dysfunction-associated steatohepatitis (MASH) in mice subjected to a Western-style high-fat, high-fructose, high-cholesterol diet. Moreover, treatment…
View article: Acyl-CoA synthetase 6 controls rod photoreceptor function and survival by shaping the phospholipid composition of retinal membranes
Acyl-CoA synthetase 6 controls rod photoreceptor function and survival by shaping the phospholipid composition of retinal membranes Open
View article: Novel Approaches for Elongation of Fish Oils into Very-Long-Chain Polyunsaturated Fatty Acids and Their Enzymatic Interesterification into Glycerolipids
Novel Approaches for Elongation of Fish Oils into Very-Long-Chain Polyunsaturated Fatty Acids and Their Enzymatic Interesterification into Glycerolipids Open
Elongation of the Very-Long-Chain Fatty Acids-4 (ELOVL4) enzyme that is expressed in neuronal tissues, sperm, and testes mediates biosynthesis of very-long-chain polyunsaturated fatty acids (VLC-PUFAs) from dietary long chain PUFAs (LC-PUF…
View article: A novel ELOVL4 variant, L168S, causes early childhood-onset Spinocerebellar ataxia-34 and retinal dysfunction: a case report
A novel ELOVL4 variant, L168S, causes early childhood-onset Spinocerebellar ataxia-34 and retinal dysfunction: a case report Open
View article: Response of the Glutathione (GSH) Antioxidant Defense System to Oxidative Injury in Necrotizing Enterocolitis
Response of the Glutathione (GSH) Antioxidant Defense System to Oxidative Injury in Necrotizing Enterocolitis Open
Necrotizing enterocolitis (NEC) is a neonatal intestinal disease associated with oxidative stress. The targets of peroxidation and the role of the innate intestinal epithelial antioxidant defense system are ill-defined. We hypothesized tha…
View article: Ablation of pigment epithelium-derived factor receptor (PEDF-R/Pnpla2) causes photoreceptor degeneration
Ablation of pigment epithelium-derived factor receptor (PEDF-R/Pnpla2) causes photoreceptor degeneration Open
Photoreceptor cells express the patatin-like phospholipase domain-containing 2 (PNPLA2) gene that codes for pigment epithelium-derived factor receptor (PEDF-R) (also known as ATGL). PEDF-R exhibits phospholipase activity that mediates the …
View article: Dietary protection against the visual and motor deficits induced by experimental autoimmune encephalomyelitis
Dietary protection against the visual and motor deficits induced by experimental autoimmune encephalomyelitis Open
Introduction Five to eight percent of the world population currently suffers from at least one autoimmune disorder. Despite multiple immune modulatory therapies for autoimmune demyelinating diseases of the central nervous system, these tre…
View article: Sperm very long-chain polyunsaturated fatty acids: relation to semen parameters and live birth outcome in a multicenter trial
Sperm very long-chain polyunsaturated fatty acids: relation to semen parameters and live birth outcome in a multicenter trial Open
View article: Transmembrane protein 135 regulates lipid homeostasis through its role in peroxisomal DHA metabolism
Transmembrane protein 135 regulates lipid homeostasis through its role in peroxisomal DHA metabolism Open
View article: ELOVL4 Mutations That Cause Spinocerebellar Ataxia-34 Differentially Alter Very Long Chain Fatty Acid Biosynthesis
ELOVL4 Mutations That Cause Spinocerebellar Ataxia-34 Differentially Alter Very Long Chain Fatty Acid Biosynthesis Open
View article: Western diet-induced shifts in the maternal microbiome are associated with altered microRNA expression in baboon placenta and fetal liver
Western diet-induced shifts in the maternal microbiome are associated with altered microRNA expression in baboon placenta and fetal liver Open
Maternal consumption of a high-fat, Western-style diet (WD) disrupts the maternal/infant microbiome and contributes to developmental programming of the immune system and nonalcoholic fatty liver disease (NAFLD) in the offspring. Epigenetic…
View article: Dietary Protection Against the Visual and Motor Deficits Induced by Experimental Autoimmune Encephalomyelitis
Dietary Protection Against the Visual and Motor Deficits Induced by Experimental Autoimmune Encephalomyelitis Open
Five to eight percent of the world population currently suffers from at least one autoimmune disorder. Despite multiple immune modulatory therapies for autoimmune demyelinating diseases of the central nervous system, these treatments can b…
View article: Western diet-induced shifts in the maternal microbiome are associated with altered microRNA expression in baboon placenta and fetal liver
Western diet-induced shifts in the maternal microbiome are associated with altered microRNA expression in baboon placenta and fetal liver Open
Maternal consumption of a high-fat, Western-style diet (WD) disrupts the maternal/infant microbiome and contributes to developmental programming of the immune system and nonalcoholic fatty liver disease (NAFLD) in the offspring. Epigenetic…
View article: Obesity promotes lipid accumulation in mouse cartilage—A potential role of acetyl‐CoA carboxylase (ACC) mediated chondrocyte de novo lipogenesis
Obesity promotes lipid accumulation in mouse cartilage—A potential role of acetyl‐CoA carboxylase (ACC) mediated chondrocyte de novo lipogenesis Open
Obesity promotes the development of osteoarthritis (OA). It is also well‐established that obesity leads to excessive lipid deposition in nonadipose tissues, which often induces lipotoxicity. The objective of this study was to investigate c…
View article: W246G Mutant ELOVL4 Impairs Synaptic Plasticity in Parallel and Climbing Fibers and Causes Motor Defects in a Rat Model of SCA34
W246G Mutant ELOVL4 Impairs Synaptic Plasticity in Parallel and Climbing Fibers and Causes Motor Defects in a Rat Model of SCA34 Open
View article: HK2 Mediated Glycolytic Metabolism in Mouse Photoreceptors Is Not Required to Cause Late Stage Age-Related Macular Degeneration-Like Pathologies
HK2 Mediated Glycolytic Metabolism in Mouse Photoreceptors Is Not Required to Cause Late Stage Age-Related Macular Degeneration-Like Pathologies Open
Age-related macular degeneration (AMD) is a multifactorial disease of unclear etiology. We previously proposed that metabolic adaptations in photoreceptors (PRs) play a role in disease progression. We mimicked these metabolic adaptations i…
View article: W246G Mutant ELOVL4 Impairs Synaptic Plasticity in Parallel and Climbing Fibers and Causes Motor Defects in a Rat Model of SCA34
W246G Mutant ELOVL4 Impairs Synaptic Plasticity in Parallel and Climbing Fibers and Causes Motor Defects in a Rat Model of SCA34 Open
Spinocerebellar ataxias (SCA) are a group of neurodegenerative disorders caused by a number of different mutations the leads to loss of motor coordination with characteristic ages of onset, symptomatology, and rates of progression. SCA typ…
View article: W246G Mutant ELOVL4 Impairs Synaptic Plasticity in Parallel and Climbing Fibers and Causes Motor Defects in a Rat Model of SCA34
W246G Mutant ELOVL4 Impairs Synaptic Plasticity in Parallel and Climbing Fibers and Causes Motor Defects in a Rat Model of SCA34 Open
Background: Spinocerebellar ataxias (SCA) are a group of neurodegenerative disorders characterized by neuronal degeneration leading to loss of motor coordination. A number of different mutations gives rise to different types of SCA with ch…
View article: Very long chain fatty acid-containing lipids: a decade of novel insights from the study of ELOVL4
Very long chain fatty acid-containing lipids: a decade of novel insights from the study of ELOVL4 Open
Lipids play essential roles in maintaining cell structure and function by modulating membrane fluidity and cell signaling. The fatty acid elongase-4 (ELOVL4) protein, expressed in retina, brain, Meibomian glands, skin, testes and sperm, is…
View article: The Elovl4 Spinocerebellar Ataxia-34 Mutation 736T>G (p.W246G) Impairs Retinal Function in the Absence of Photoreceptor Degeneration
The Elovl4 Spinocerebellar Ataxia-34 Mutation 736T>G (p.W246G) Impairs Retinal Function in the Absence of Photoreceptor Degeneration Open
View article: Phosphoinositide Lipids in Ocular Tissues
Phosphoinositide Lipids in Ocular Tissues Open
Inositol phospholipids play an important role in cell physiology. The inositol head groups are reversibly phosphorylated to produce seven distinct phosphorylated inositides, commonly referred to as phosphoinositides (PIs). These seven PIs …
View article: Altered photoreceptor metabolism in mouse causes late stage age-related macular degeneration-like pathologies
Altered photoreceptor metabolism in mouse causes late stage age-related macular degeneration-like pathologies Open
Significance The main cause for blindness in the elderly worldwide is age-related macular degeneration (AMD). What causes AMD remains unknown. The high metabolic demands of photoreceptors are thought to contribute to disease pathogenesis, …
View article: Decreased very long chain polyunsaturated fatty acids in sperm correlates with sperm quantity and quality
Decreased very long chain polyunsaturated fatty acids in sperm correlates with sperm quantity and quality Open
View article: Differential composition of DHA and very-long-chain PUFAs in rod and cone photoreceptors
Differential composition of DHA and very-long-chain PUFAs in rod and cone photoreceptors Open
View article: Pyruvate kinase M2 regulates photoreceptor structure, function, and viability
Pyruvate kinase M2 regulates photoreceptor structure, function, and viability Open
View article: Elovl4 5-bp deletion does not accelerate cone photoreceptor degeneration in an all-cone mouse
Elovl4 5-bp deletion does not accelerate cone photoreceptor degeneration in an all-cone mouse Open
Mutations in the elongation of very long chain fatty acid 4 (ELOVL4) gene cause Stargardt macular dystrophy 3 (STGD3), a rare, juvenile-onset, autosomal dominant form of macular degeneration. Although several mouse models have already been…
View article: Homozygous Expression of Mutant ELOVL4 Leads to Seizures and Death in a Novel Animal Model of Very Long-Chain Fatty Acid Deficiency
Homozygous Expression of Mutant ELOVL4 Leads to Seizures and Death in a Novel Animal Model of Very Long-Chain Fatty Acid Deficiency Open
View article: Regional changes in CNS and retinal glycerophospholipid profiles with age: a molecular blueprint
Regional changes in CNS and retinal glycerophospholipid profiles with age: a molecular blueprint Open
View article: Loss of human disease protein retinitis pigmentosa GTPase regulator (RPGR) differentially affects rod or cone-enriched retina
Loss of human disease protein retinitis pigmentosa GTPase regulator (RPGR) differentially affects rod or cone-enriched retina Open
It is unclear how genes, such as RPGR (retinitis pigmentosa guanine triphosphatase regulator) that are expressed in both rods and cones, cause variable disease pathogenesis. Using transcriptomic analysis, we show that loss of RPGR in a rod…
View article: PBN (Phenyl-N-Tert-Butylnitrone)-Derivatives Are Effective in Slowing the Visual Cycle and Rhodopsin Regeneration and in Protecting the Retina from Light-Induced Damage
PBN (Phenyl-N-Tert-Butylnitrone)-Derivatives Are Effective in Slowing the Visual Cycle and Rhodopsin Regeneration and in Protecting the Retina from Light-Induced Damage Open
A2E and related toxic molecules are part of lipofuscin found in the retinal pigment epithelial (RPE) cells in eyes affected by Stargardt's disease, age-related macular degeneration (AMD), and other retinal degenerations. A novel therapeuti…