• The protein encoded by this gene is a phosphatidylglycerol remodeling protein found at the interface of mitochondria and endoplasmic reticula, where it mediates phospholipid exchange. (wikipedia.org)
  • The mitochondrial membrane contains the major classes of phospholipids found in all cell membranes, including phosphatidylcholine (PC), phosphatidylethanolamine (PE), phosphatidylinositol (PI), phosphatidylserine (PS), and phosphatidic acid (PA), as well as phosphatidylglycerol (PG), the precursor for cardiolipin (CL), which is predominantly located in the mitochondria. (lifestylematrix.com)
  • Here, we demonstrate that in a niche-like co-culture system, AML cells took up functional mitochondria from bone marrow stromal cells (BMSCs) and inhibition of such mitochondrial transfer by metformin, the most commonly prescribed drug for type 2 diabetes mellitus, significantly enhanced the chemosensitivity of AML cells co-cultured with BMSCs. (biomed.news)
  • Mammalian respiratory complex I (CI) is a 45-subunit, redox-driven proton pump that generates an electrochemical gradient across the mitochondrial inner membrane to power ATP synthesis in mitochondria. (biomed.news)
  • Phosphatidate can be converted to CDP-diacylglycerol which is the precursor for the acidic phospholipids, phosphatidylglycerol, cardiolipin and phosphatidylinositol, or it can be converted to diacylglycerol (DG) by dephosphorylation. (aocs.org)
  • Hippocampal lipids were not significantly affected by APOE genotype, however levels of myelin-enriched sulfatides and hexosylceramides were significantly lower, and polyunsaturated phospholipids were higher, in rs1990622-A carriers after controlling for APOE genotype. (biomedcentral.com)
  • Initially these disorders were named according to symptoms (later the cause was found to be mitochondrial dysfunction and more specifically maternally inherited mitochondrial DNA variations). (medlink.com)
  • 1 Many conditions lead to fatigue, but mitochondrial dysfunction is at the core. (lifestylematrix.com)
  • Barth syndrome (BTHS) is a severe disorder, due to dysfunction of the mitochondrial cardiolipin acyl transferase tafazzin. (bvsalud.org)
  • Membrane lipid replacement (MLR) is best utilized in the form of an oral phospholipid supplement that emulates the composition of the mitochondrial lipids. (lifestylematrix.com)
  • By effectively replacing these membrane lipids, mitochondrial function is optimized, and energy production is restored to optimal levels using a safe and cost-effective intervention. (lifestylematrix.com)
  • Our study demonstrates that TMEM106B protein abundance is increased with brain ageing in humans, establishes that dementia risk allele rs1990622-A predisposes to TMEM106B fibril formation in the hippocampus, and provides the first evidence that rs1990622-A affects brain lipid homeostasis, particularly myelin lipids. (biomedcentral.com)
  • Lipids are not only a major factor influencing the development and processing of food products, but also affect human health. (creative-proteomics.com)
  • For example, lipids affect the texture, rheological properties, digestibility and storage stability of cereals and cereal products, and the lipid composition of cereals is closely related to the origin. (creative-proteomics.com)
  • Changes in lipids can affect changes in food quality and thus food safety. (creative-proteomics.com)
  • The resulting CDP-diacylglycerol can be utilized immediately for the synthesis of phosphatidylglycerol (PG), and thence cardiolipin (CL), and of phosphatidylinositol (PI). (ymdb.ca)
  • Loss of prM-cholesterol association has a bipartite effect reducing ZIKV entry and leading to assembly defects. (bvsalud.org)
  • From the formula it appears clearly that phosphatidylserine possesses three ionizable groups: a diester phosphoric acid, an amino group and a carboxyl function. (gerli.com)
  • The encoded protein plays a major role in mitochondrial function and intracellular cholesterol trafficking. (wikipedia.org)
  • The SERAC1 gene encodes for a protein necessary for phosphatidylglycerol remodeling. (wikipedia.org)
  • The protein may also be involved in the transacylation-acylation reaction to produce phosphatidylglycerol-36:1 and bis(monoacylglycerol)phosphate biosynthetic pathway. (wikipedia.org)
  • The SERAC1 gene mutations that cause this condition reduce the amount of SERAC1 protein that is produced or lead to production of a protein with little or no function. (wikipedia.org)
  • We identify mitoguardin-2, a mitochondrial protein at contacts with the ER and/or lipid droplets (LDs), as a lipid transporter. (rupress.org)
  • Our data unravel a key function for lysosomal phosphoinositide metabolism in rewiring organellar membrane dynamics in response to cellular nutrient status. (bvsalud.org)
  • Because of cardiolipin, the remodeling process of phosphatidylglycerol is essential for mitochondrial function and intracellular cholesterol trafficking. (wikipedia.org)
  • Mutations in the SERAC1 gene have been associated to impairment of both mitochondrial function and intracellular cholesterol trafficking. (wikipedia.org)
  • We show here that lysosome morphology and function are reversibly controlled by a nutrient-regulated signaling lipid switch that triggers the conversion between peripheral motile mTOR complex 1 (mTORC1) signaling-active and static mTORC1-inactive degradative lysosomes clustered at the cell center. (bvsalud.org)
  • The functions of the lipins in co-regulating the effects of PGC-1α and PPARα and also acting as a PAP demonstrate the unique abilities of lipins to regulate both glycerolipid synthesis and fatty acid oxidation. (aocs.org)
  • As a result, phosphatidylglycerol remodeling is impaired, which likely alters the composition of cardiolipin. (wikipedia.org)
  • Now, a variety of genes that cause mitochondrial disease have been discovered. (medlink.com)
  • PGC-1α and PPARα are both master regulators of genes involved in mitochondrial biogenesis and fatty acid oxidation, respectively [reviewed in 6]. (aocs.org)
  • ANOVA was used to test the effect of major dementia risk alleles in the TMEM106B and APOE genes on the hippocampal proteome and lipidome, adjusting for age, gender, and post-mortem interval. (biomedcentral.com)
  • Our data suggests that TMEM106B is one of a growing list of major dementia risk genes that affect glial lipid metabolism. (biomedcentral.com)
  • The most common and evolutionarily conserved functions of these organelles include the β-oxidation of fatty acids and detoxification of H 2 O 2 [2]. (5dok.net)
  • Categorizing and describing a mitochondrial disorder is complicated in part because there are 3 ways to do so. (medlink.com)
  • Mitochondrial disorders describe a broad range of disorders that have multiple genetic causes and variable symptoms and signs. (medlink.com)
  • In complexes, the other enzymes affect cleaved CBF1( or RBPJkappa), while in channels they are required Lag-1, immediately that the m ' CSL ' demonstrates separated been to this produced feature experience breast. (evakoch.com)
  • Here, we employed a genetically encoded high-affinity H2 O2 sensor, HyPer7, in mammalian tissue culture cells to investigate different modes of mitochondrial H2 O2 release. (biomed.news)
  • Thus at pH 7, the phosphate and the carboxyl functions are in anionic form and the amino group is positively charged. (gerli.com)
  • TMEM106B, a regulator of lysosomal and oligodendrocyte function, was regulated with greatest effect size. (biomedcentral.com)
  • Researchers speculate that the abnormal cardiolipin affects mitochondrial function, reducing cellular energy production and leading to the neurological and hearing problems characteristic of MEGDEL syndrome. (wikipedia.org)
  • In general, mitochondrial disorders are disorders of energy production. (medlink.com)
  • This study aimed to determine how ageing and major genetic risk factors for dementia affect the hippocampal proteome and lipidome of neurologically-normal humans over the age of 65. (biomedcentral.com)
  • Lysosomes serve dual antagonistic functions in cells by mediating anabolic growth signaling and the catabolic turnover of macromolecules. (bvsalud.org)
  • In addition, metformin potentiated the antitumor efficacy of cytarabine (Ara-C) in vivo in an NCG immunodeficient mouse xenograft model by inhibiting the mitochondrial transfer and OXPHOS activity in the engrafted human AML cells. (biomed.news)
  • Altogether, this study identifies a potential application of metformin in sensitizing AML cells to chemotherapy and unveils a novel mechanism by which metformin executes such effect via blocking the mitochondrial transfer from stromal cells to AML cells. (biomed.news)
  • In this review of what is probably the most heterogeneous group of human diseases (the mitochondrial diseases including encephalomyopathies), the author tries to offer the clinician a rational diagnostic framework. (medlink.com)
  • In such biomolecules the distinctive chemical and physical proper-ties of their components are blended to fill specialized bio-logical functions. (medmuv.com)
  • Cardiolipin is responsible for converting energy acquired from food to a cell-usable form and required for proper mitochondrial function. (wikipedia.org)
  • Their function, number and shape vary depending on the organism, cell type and the environmental conditions. (5dok.net)