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Human ACADVL Protein expressed in Wheat germ - ABIN1305595
Muroya, Ito, Rong, Takashima, Ito, Cao, Nakamura, Joh, Kohzuki: Disorder of fatty acid metabolism in the kidney of PAN-induced nephrotic rats. in American journal of physiology. Renal physiology 2012
There are currently no biochemical markers for prediction of disease severity and for the need for treatment in VLCAD deficiency. Mutation analysis may offer predictive value but this may not be robust enough for a large proportion of those mutations that have not been previously reported in clinically affected patients.
We retrospectively analyzed early outcomes for individuals who were diagnosed with VLCAD deficiency by NBS (show NBN Proteins) and describe initial presentations, diagnosis, clinical outcomes and treatment in a cohort of 52 individuals ages 1-18year.
LCHAD (show HADHA Proteins) and MCAD are differentially expressed in maternal and fetal tissues during normal late pregnancy, which may represent a metabolic adaptation in response to physiological maternal dyslipidemia during late pregnancy.
11 mutations in ACADVL gene in 7 patients, 7 reported (p.S22X, p.W427X, p.A213T, p.G222R, p.R450H, c.296-297delCA, c.1605+1G>T), 4 novel (p.S72F, p.Q100X, p.M437T, p.D466Y). p.R450H and p.D466Y (14.28%, 2/14 alleles) mutations identified in 2 alleles.
Case Report: missense mutation within the ACADVL gene responsible for very-long-chain acyl-CoA dehydrogenase deficiency and sudden infant death.
These results emphasize the importance of functional investigation of abnormal NBS (show NBN Proteins) or clinical testing suggestive but not diagnostic of very-long-chain acyl-CoA dehydrogenase .
These findings support the importance of considering that mutations may be present in the ACADVL gene when a significant partial deficiency is found in CPTII (show CPT2 Proteins) activity, but no mutations in the CPT2 (show CPT2 Proteins) gene can be identified.
Identification of 2 VLCAD mutations leads to precautions in the management of the children with VLCAD deficiency.
The expressions of LCHAD (show HADHA Proteins) gene and protein are remarkably reduced in early onset severe preeclampsia and HELLP syndrome.
Analyzed potential rhabdomyolysis-susceptibility genes (RYR 1 (show RYR1 Proteins), CPT II (show CPT2 Proteins), VLCAD and CYP (show PPIG Proteins) 2D6) from autopsy samples of methamphetamine abusers; no obvious relationship between the genetic mutations observed in this study and rhabdomyolysis was seen.
Triheptanoin was not able to prevent the development of systolic dysfunction in VLCAD(-/-) mice despite an upregulation of cardiac glucose oxidation. Strikingly, the anaplerotic effects of triheptanoin were restricted to the liver.
observed strong upregulation of peroxisomal beta-oxidation in VLCAD(-/-) mice
SIRT3 (show SIRT3 Proteins) and SIRT5 (show SIRT5 Proteins) regulate the enzyme activity and cardiolipin binding of very long-chain acyl-CoA dehydrogenase
Studies conducted with permeabilized mitochondria and different chain length acyl-CoA (show GNPAT Proteins) derivatives suggest that VLCAD is also a source of reactive oxygen species production in mitochondria of high fat diet animals.
We demonstrate here that both dietary interventions with respect to the fat content of the diet reverse endogenous compensatory mechanisms in muscle that have evolved in VLCAD(-/-) mice resulting in pronounced energy deficiency
VLCAD(-/-) mice develop tissue-specific strategies to compensate deficiency of VLCAD either by induction of other mitochondrial acyl-CoA (show GNPAT Proteins) dehydrogenases or by enhancement of glucose oxidation.
Report a longer QTc interval and lipid alterations in VLCAD null mice.
Four VLCAD-/- deficient mice died unexpectedly on the treadmill during the early stages of training. The VLCAD-/- deficient mice that survived adapted to the aerobic interval training similarly to the non-deficient mice.
Medium-chain triglycerides impair lipid metabolism and induce hepatic steatosis in very long-chain acyl-CoA dehydrogenase (VLCAD)-deficient mice
Data show that in VLCAD knockout mice fed a long-chain triglyceride diet, fasting results in accumulation of liver lipids, hepatopathy and upregulation of peroxisomal and microsomal oxidation pathways as well as antioxidant enzyme activities and TBARS.
Bovine ACADVL gene had a significant effect on chest width (P<0.05), chest depth (P<0.05), and hip width (P<0.05) in the Qinchuan breed.
The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms.
acyl-Coenzyme A dehydrogenase, very long chain
, very long-chain specific acyl-CoA dehydrogenase, mitochondrial
, VLCAD very-long-chain acyl-CoA dehydrogenase
, Very long chain Acyl-Coa dehydrogenase
, acyl-coenzyme A dehydrogenase, very long chain
, Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
, Very-long-chain acyl-CoA dehydrogenase