The enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results.
about
Effect of Dietary Restriction and Subsequent Re-Alimentation on the Transcriptional Profile of Bovine Skeletal MuscleClinical, biochemical and metabolic characterisation of a mild form of human short-chain enoyl-CoA hydratase deficiency: significance of increased N-acetyl-S-(2-carboxypropyl)cysteine excretionLong-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunctionFatty acid omega-oxidation as a rescue pathway for fatty acid oxidation disorders in humans.Risk stratification by residual enzyme activity after newborn screening for medium-chain acyl-CoA dehyrogenase deficiency: data from a cohort study.Acyl-CoA thioesterase 9 (ACOT9) in mouse may provide a novel link between fatty acid and amino acid metabolism in mitochondria.Heterogeneous N-terminal acylation of retinal proteins results from the retina's unusual lipid metabolismThe functions of cardiolipin in cellular metabolism-potential modifiers of the Barth syndrome phenotypeThe domain-specific and temperature-dependent protein misfolding phenotype of variant medium-chain acyl-CoA dehydrogenaseAcadl-SNP based genotyping assay for long-chain acyl-CoA dehydrogenase deficient miceIn vitro and in vivo consequences of variant medium-chain acyl-CoA dehydrogenase genotypesRecurrent Ventricular Tachycardia in Medium-Chain Acyl-Coenzyme A Dehydrogenase DeficiencyThe Newborn Screening Paradox: Sensitivity vs. Overdiagnosis in VLCAD DeficiencyMitochondrial-associated metabolic disorders: foundations, pathologies and recent progress.NOX4-dependent fatty acid oxidation promotes NLRP3 inflammasome activation in macrophages.Mitochondrial dysfunction and oxidative stress in aging and cancer.Pathophysiology of fatty acid oxidation disorders and resultant phenotypic variability.Lipid metabolism in mitochondrial membranes.Evolution of peroxisomes illustrates symbiogenesis.Mitochondrial trifunctional protein deficiency in human cultured fibroblasts: effects of bezafibrate.Lethal neonatal case and review of primary short-chain enoyl-CoA hydratase (SCEH) deficiency associated with secondary lymphocyte pyruvate dehydrogenase complex (PDC) deficiency.Riboflavin Responsive Mitochondrial Dysfunction in Neurodegenerative Diseases.Muscle MRI in patients with long-chain fatty acid oxidation disorders.The Peroxisome-Mitochondria Connection: How and Why?Dysregulation of hepatic cAMP levels via altered Pde4b expression plays a critical role in alcohol-induced steatosis.Genetic dissection in a mouse model reveals interactions between carotenoids and lipid metabolism.A method for measuring fatty acid oxidation in C. elegans.Development of a Tandem Mass Spectrometry Method for Rapid Measurement of Medium- and Very-Long-Chain Acyl-CoA Dehydrogenase Activity in FibroblastsThe lauric acid-activated signaling prompts apoptosis in cancer cells.A compendium of inborn errors of metabolism mapped onto the human metabolic network.Necrotizing enterocolitis and respiratory distress syndrome as first clinical presentation of mitochondrial trifunctional protein deficiency.Localization of aristolochic acid in mouse kidney tissues by immunohistochemistry using an anti-AA-I and AA-II monoclonal antibody.Non-invasive test using palmitate in patients with suspected fatty acid oxidation defects: disease-specific acylcarnitine patterns can help to establish the diagnosis.Infant with hepatomegaly and hypoglycemia: A setting for fatty acid oxidation defects.Fatty acid oxidation flux predicts the clinical severity of VLCAD deficiency.Clinical and molecular investigation of 14 Japanese patients with complete TFP deficiency: a comparison with Caucasian cases.
P2860
Q27321363-184FA9B5-2F2D-4B97-BDF7-52C6BB9DD8C7Q28118617-0979139A-5AD1-44D8-BA96-2A334306E384Q28308215-DE4BA7CD-84ED-4D20-9BEF-DEB3674D3875Q34025017-A6ABCCBA-F5C1-47C7-A57D-F9D33BBAF600Q34282546-2E3BDF22-31B5-411B-ABC2-1C61244BA3DCQ34358136-1FC79A4D-4D6F-423A-9703-633348694594Q34917148-E58ECD1D-4CD7-420D-A226-7F49576949FBQ35128445-0A0DAF70-B469-4471-8C4E-B24EE563350EQ35144068-97E46F59-1315-48B0-9A7F-E6A81DBBEFC3Q35911338-A5EF870D-228C-46A7-9909-CAC0969610A1Q36722478-C998030D-2E14-4F17-A1C1-98049C034DF8Q36894752-D81294F3-937F-44D5-9DB3-53FA1F1C8CC3Q36894924-64E9FBD3-773D-4925-A8BD-BF910EF54377Q37369860-E3FAA12A-38E1-424A-AC97-C17BC3E706F1Q37548406-03C059DD-3F38-4E78-8FE1-B6091BFA437AQ37564295-3533E025-C609-47D2-B09E-F29E5AC4DDC5Q38106893-432F0E26-D66B-4AEB-835A-1C50389FE6E9Q38235898-D981DA3D-0413-4AD6-9CFB-ED8835DEF3E3Q38637715-C47B9A93-D6A0-41CD-BA1C-4A11254D4D93Q38860352-2E2A419A-1B54-4911-8933-027D99BCC1A7Q39137114-DE2871E4-A881-4E74-84DD-707A0868CFF2Q39285651-89500538-1A2B-4966-AAD2-4FE0ECF9E119Q39301323-C402A2EE-6800-4992-8664-6459E989E71DQ39326795-7E443353-0659-40E5-BF22-848CF58254F7Q39692028-B37B5A9E-78CC-4716-BE90-72592928E2A2Q40965992-D68BA15F-9DD4-480C-AFCE-0D5BF6CDC3EBQ41383148-AABFF152-6376-44E3-A87B-659B2CADE8F1Q41619196-D4B55BCD-DDD9-4E44-ACC6-C3A60A8D039BQ41724625-741A4C58-2D76-4B0A-9E43-AFDC55F72349Q42653560-2BA189C1-C5EC-4A02-A7D2-6E2DE2DB257EQ43245541-92E19713-D27E-46C4-BBD0-E75C108A29BAQ46810023-BFC941C6-A9A3-4FF4-A4D0-C7E68025D083Q47147365-EB89426F-F4D7-4C30-A9B9-9D5EAE6D34D7Q48126558-141C4F69-F067-4765-901F-41F17B6A4D7DQ48207318-353D2765-4965-4803-A80F-EE9BA024787CQ50995774-CB907CE2-CEFD-4D47-8406-E44F28FAEACB
P2860
The enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on 20 May 2010
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
The enzymology of mitochondria ...... ve neonatal screening results.
@en
The enzymology of mitochondria ...... ve neonatal screening results.
@nl
type
label
The enzymology of mitochondria ...... ve neonatal screening results.
@en
The enzymology of mitochondria ...... ve neonatal screening results.
@nl
prefLabel
The enzymology of mitochondria ...... ve neonatal screening results.
@en
The enzymology of mitochondria ...... ve neonatal screening results.
@nl
P2093
P2860
P1476
The enzymology of mitochondria ...... ve neonatal screening results.
@en
P2093
Hans R Waterham
Jos P N Ruiter
Lodewijk IJLst
P2860
P2888
P304
P356
10.1007/S10545-010-9104-8
P577
2010-05-20T00:00:00Z