oalib

OALib Journal期刊

ISSN: 2333-9721

费用：99美元

投稿

时间不限

( 2026 )

( 2025 )

( 2024 )

( 2022 )

自定义范围…

匹配条件： “Carnitine palmitoyl transferase” ，找到相关结果约628条。
列表显示的所有文章，均可免费获取
第1页/共628条
每页显示 条
Case Report: Carnitine Palmitoyl Transferase II (CPT II) Deficiency  [PDF]
Kathy Po, Marius Chivu, Edwin Rosas, Balpreet Kaur
Open Journal of Internal Medicine (OJIM) , 2024, DOI: 10.4236/ojim.2024.141009
Abstract: Carnitine Palmitoyl Transferase II (CPTII) is a very important enzyme that helps with the oxidation of long-chain fatty acid to produce energy. Deficiency in CPTII will lead to energy deficiency in the case of fasting and the accumulation of the long chain fatty in the body. There are three types of CPT II deficiency, the myopathic form, the severe infantile hepatocardiomuscular form and the lethal neonatal form. They are all inherited as an autosomal recessive. Diagnosis of the CPTII are 1) tandem mass spectrometry (MS/MS) in adult form and 2) CPTII polymorphism (F352C), which is linked to reducing the activity of CPTII in infantile form [1]. Glucose is the primary management and medium-chain fatty acid is an alternative due to the bypass of the CPTII enzyme in the pathway. For the prevention of CPTII deficiency are to avoid long chain fatty acid (C12-fatty acid), fasting, prolonged exercise, known triggers, and certain medications such as anti-epileptics and general anesthesia. During the rhabdomyolysis and myoglobinuria attack, it is very important to maintain hydration to avoid acute renal failure. If, however, renal failure occurs, dialysis is recommended. We present a case of a 27-year-old African American woman with the significant past medical history of CPT II deficiency leading to recurrent rhabdomyolysis and myoglobinuria. Together with all the research studies from diagnosis to treatment of CPTII deficiency will help in clinical management of patients. And this case report will add to the existing case reports of patients who have CPTII deficiency in terms of how we diagnose, how we treat, and how we prevent symptoms from re-occurring.
Effects of high-intensity intermittent training on carnitine palmitoyl transferase activity in the gastrocnemius muscle of rats
Carnevali Jr, L.C.;Eder, R.;Lira, F.S.;Lima, W.P.;Gon？alves, D.C.;Zanchi, N.E.;Nicastro, H.;Lavoie, J.M.;Seelaender, M.C.L.;
Brazilian Journal of Medical and Biological Research , 2012, DOI: 10.1590/S0100-879X2012007500105
Abstract: we examined the capacity of high-intensity intermittent training (hi-it) to facilitate the delivery of lipids to enzymes responsible for oxidation, a task performed by the carnitine palmitoyl transferase (cpt) system in the rat gastrocnemius muscle. male adult wistar rats (160-250 g) were randomly distributed into 3 groups: sedentary (sed, n = 5), hi-it (n = 10), and moderate-intensity continuous training (mi-ct, n = 10). the trained groups were exercised for 8 weeks with a 10% (hi-it) and a 5% (mi-ct) overload. the hi-it group presented 11.8% decreased weight gain compared to the sed group. the maximal activities of cpt-i, cpt-ii, and citrate synthase were all increased in the hi-it group compared to the sed group (p < 0.01), as also was gene expression, measured by rt-pcr, of fatty acid binding protein (fabp; p < 0.01) and lipoprotein lipase (lpl; p < 0.05). lactate dehydrogenase also presented a higher maximal activity (nmol·min-1·mg protein-1) in hi-it (around 83%). we suggest that 8 weeks of hi-it enhance mitochondrial lipid transport capacity thus facilitating the oxidation process in the gastrocnemius muscle. this adaptation may also be associated with the decrease in weight gain observed in the animals and was concomitant to a higher gene expression of both fabp and lpl in hi-it, suggesting that intermittent exercise is a "time-efficient" strategy inducing metabolic adaptation.
7. Carnitine: A novel health factor-An overview
C.D. Dayanand,N. Krishnamurthy,S. Ashakiran,K.N. Shashidhar
International Journal of Pharmaceutical and Biomedical Research (IJPBR) , 2011,
Abstract: Carnitine term comprises L-carnitine, acetyl –L-carnitine and Propionyl –L-carnitine. Carnitine in greater amount obtained from animal dietary sources as compared to plant sources. The endogenous synthesis of carnitine takes place in animal tissues like liver, kidney and brain using precursor amino acid lysine and methionine by iron, vitamin C, niacin, pyridoxine dependent pathway. This is the basis of vegans generally depending on carnitine in larger proportion through in vivo synthesis than omnivorous subjects. The concentration of Tri methyl lysine residues and the tissue specificity of Butyro betaine Dehydrogenase will play a significant role in regulating the carnitine biosynthesis. Carnitine transport from the site of synthesis to target tissue occurs via blood. Therefore, the measurement of normal plasma carnitine concentration represents the balance between the rate of synthesis and rate of excretion through specific transporter proteins. The cellular functional role of carnitine depends on the uptake in to cells through carnitine transport proteins and transport in to mitochondrial matrix. The function of carnitine is to traverse Long chain Fatty Acids across inner mitochondrial membrane for β-oxidation for rapid production of ATP. The carnitine level in plasma or tissue is done by spectro photometric, HPLC, or Tandem Mass Spectro photometry methods. Carnitine deficiency results in muscle disorders, there are two types of deficiency states such as primary and secondary deficiency. The primary is of systemic or myopathic, characterized by defect of high affinity organic cation transporter protein present on the plasma membrane of liver and kidney and also due to dysfunction of carnitine reabsorbtion through similar transport proteins in renal tubules. However, secondary carnitine deficiency associated with mitochondrial disorders and also defect of β-oxidation such as CPT-II and acyl CoA Dehydrogenase, several other causes are vitamin c deficiency, valaproate therapy, fancony syndrome, liver dysfunction, and kidney disease. Both these conditions results in recurrent muscle cramp, muscle weakness and fatigue, non ketotic hypoglycemia, encephalopathy, hepatomegaly, muscle necrosis, etc. Therefore, In view of the life threatening events of carnitine deficiency, Food drug administration considered L-carnitine as a drug to treat the primary and secondary carnitine deficiency. In recent times, carnitine has been extensively studied in various research activities to explore the therapeutic benefit. Thus, carnitine justifies as a novel health factor.
Cyanidin-3-O-β-glucoside regulates fatty acid metabolism via an AMP-activated protein kinase-dependent signaling pathway in human HepG2 cells
Honghui Guo, Guoling Liu, Ruimin Zhong, Yun Wang, Duan Wang, Min Xia
Lipids in Health and Disease , 2012, DOI: 10.1186/1476-511x-11-10
Abstract: Anthocyanin Cy-3-g increased cellular AMPK activity in a calmodulin kinase kinase dependent manner. Furthermore, Cy-3-g substantially induced AMPK downstream target ACC phosphorylation and inactivation, and then decreased malonyl CoA contents, leading to stimulation of CPT-1 expression and significant increase of fatty acid oxidation in HepG2 cells. These effects of Cy-3-g are largely abolished by pharmacological and genetic inhibition of AMPK.This study demonstrates that Cy-3-g regulates hepatic lipid homeostasis via an AMPK-dependent signaling pathway. Targeting AMPK activation by anthocyanin may represent a promising approach for the prevention and treatment of obesity-related nonalcoholic fatty liver disease.Nonalcoholic fatty liver disease (NAFLD) is a serious consequence of obesity, increasing the risk of liver cancer or cirrhosis [1]. The origin of this disease is unknown and probably multifactorial. Nevertheless, because impaired lipid metabolism is recognized as an associate and/or promoting mediator of the disease, management of hepatic metabolic disorders becomes an essential strategy for prevention and treatment of obesity-related NAFLD [2].AMP-activated protein kinase (AMPK) is a key sensor of cellular energy status and it is also recognized as a major regulator of liver and whole body lipid homeostasis [3]. AMPK activation in the liver results in the phosphorylation and inactivation of acetyl-CoA carboxylase (ACC), a direct AMPK substrate, leading to decreased conversion of acetyl-CoA to malonyl CoA [4]. AMPK activation also results in phosphorylation and activation of malonyl CoA decarboxylase (MCD), resulting in further lowering of malonyl CoA levels. Malonyl CoA allosterically inhibits carnitine palmitoyl-CoA transferase 1 (CPT-1), the enzyme responsible for transport of long chain acyl-CoAs into mitochondria for oxidation. Additionally, as malonyl CoA is required for de novo synthesis of fatty acids, decreased malonyl CoA leads to a reduction in he
Mitochondrial carnitine palmitoyl transferase-II inactivity aggravates lipid accumulation in rat hepatocarcinogenesis
Deng-Bing Yao,Deng-Fu Yao,Jie Yang,Juan-Juan Gu,Li Wang,Min Yao,Wen-Jie Zheng,Yin Cai
- , 2017, DOI: 10.3748/wjg.v23.i2.256
Abstract:
Epistructured catechins, EGCG and EC facilitate apoptosis induction through targeting de novo lipogenesis pathway in HepG2 cells
Dumrongsak Pekthong,Lysiane Richert,Pattamaphron Phunsomboon,Phuriwat Khiewkamrop,Piyarat Srisawang
- , 2018, DOI: 10.1186/s12935-018-0539-6
Abstract: The cytotoxic effects of EGCG and EC on HepG2 cells and primary hepatocytes. Cells were treated with EGCG and EC at indicated dose and times. Cells incubated with 0.1% DMSO without EGCG or EC were identified as the control. Cell viability was assessed by MTT assay. a, b Cell viability of HepG2 cells and c HepG2 cells and primary hepatocytes were treated with EGCG or EC at IC50 concentration for 72 h. Data from at least three independent triplicated experiments are presented as mean？±？SD, n？=？9, *p？<？0.05, denoting significant differences compared with the contro
The Role of Neonatal Carnitine Palmitoyl Transferase Deficiency Type II on Proliferation of Neuronal Progenitor Cells and Layering of the Cerebral Cortex in the Developing Brain
Heepeel Chang,Phyllis Faust
Columbia Undergraduate Science Journal , 2007,
Abstract: Neonatal Carnitine Palmitoyl Transferase Deficiency Type II, characterized by the absence of CPT II enzyme, is one of the lethal disorders of mitochondrial fatty acid oxidation. CPT II regulates the conversion of long chain fatty acids, so that its product, acyl-CoA esters, can enter the Krebs cycle and generate energy. Neonatal mutations of CPT II lead to severe disruption of the metabolism of long-chain fatty acids and result in dysmorphic features, cystic renal dysplasia, and neuronal migration defects. Examination of the brain from an approximately 15-week gestation human fetus with CPT II deficiency revealed premature formation of cerebral cortical gyri and sulci and significantly lower levels of neuronal cell proliferation in the ventricular and subventricular zones as compared to the reference cases. We used immunohistochemical markers to further characterize the effect of CPT II deficiency on progenitor cell proliferation and layering of neurons. These studies demonstrated a premature generation of layer 5 cortical neurons. In addition, both the total number and percentage of progenitor cells proliferating in the ventricular zone were markedly reduced in the CPT II case in comparison to a reference case. Our results indicate that CPT II deficiency alters the normal program of cellular proliferation and differentiation in the cortex, with early differentiation of progenitor cells associated with premature cortical maturation.
Acute aflatoxin B1 – Induced hepatotoxicity alters gene expression and disrupts lipid and lipoprotein metabolism in rats
Anthonia Obhio Abiodun,Boluwaji Oluwamayowa Oyeniyi,Chibueze Uchechukwu Duru,Francis Adedayo Faduyile,Ogheneworo Joel Ebebeinwe,Oluwakemi Anuoluwapo Rotimi,Solomon Oladapo Rotimi
- , 2017, DOI: 10.1016/j.toxrep.2017.07.006
Abstract:
The Flow of Information from Nucleus to Golgi Is Contingent upon Nuclear Membrane Synthesis and Protraction of the Ceramide-Containing Membrane to Endoplasmic Reticulum  [PDF]
Amalia Slomiany, Bronislaw L. Slomiany
Advances in Biological Chemistry (ABC) , 2018, DOI: 10.4236/abc.2018.83005
Abstract: The nucleus-initiated augmentation of ER membrane is reflected in a coordinated synthesis and intercalation of the explicit proteins and lipids required for the replacement, repair and function of the cell and its organelles. The direct connection between nucleus and the membranes containing labeled sphingosine (SphN) and ceramide (Cer) was affirmed by determining synthetic activity of serine palmitoyltransferase (SPT). The SPT and the newly synthesized serine-labeled lipid products were identified in the Outer- and Inner-Nuclear Membrane (ONM, INM) and ER. The pulse-chase experiments disclosed that the incorporation of radiolabeled lipids into both nuclear membranes declined upon their simultaneous increase in Endoplasmic Reticulum (ER). These results, and prior findings regarding metabolic transfer of nuclear membrane phosphoinositides to the outer leaflet of ER [Slomiany and Slomiany, Health, 2011, 3, 187-199], allowed us to reason that INM and ONM are not distinct entities, but uninterrupted continuum facing nucleosol and then cytosol when protracted into segment known as ER. Consequently, the identification of SPT and its products in the inner leaflet of nuclear and ER microsomes lent credence to the luminal presence of Cer in Golgi, luminal synthesis of glycosphingolipids (GSphLs), sphingomyelin (SM), and their delivery to the outer leaflet of apical and basolateral cell membrane, respectively. The findings presented in this communication provide further support to our concept that the factual intercalation of proteins and lipids into the cell membranes can only take place during their simultaneous synthesis that is guided by the nuclear and cytosolic processes enacted in nuclear-ER membrane continuum. At the nuclear stage, the signal-specific genes expression promotes active synthesis and intercalation of lipids into the organelles’ customized membrane that is protracted and articulated in ER in form of transport vesicles.
Sodium sulfite promotes the assembly and secretion of very low-density lipoprotein in HL-7702 hepatocytes
Chunyan Zhao,Dandan Yan,Jianying Bai,Peiyu Lei,Shuyun Yang,Youping Wang
- , 2016, DOI: 10.1016/j.toxrep.2015.12.005
Abstract:
第1页/共628条
每页显示 条


Home
Copyright © 2008-2020 Open Access Library. All rights reserved.