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3-hydroxyacyl-coa dehydrogenase deficiency
A novel HPLC-based method to diagnose peroxisomal D-bifunctional protein enoyl-CoA hydratase deficiency.
3-hydroxyisobutyryl-coa hydrolase deficiency
ECHS1 mutations in Leigh disease: a new inborn error of metabolism affecting valine metabolism.
3-hydroxyisobutyryl-coa hydrolase deficiency
Whole-exome sequencing identifies novel ECHS1 mutations in Leigh syndrome.
acetyl-coa c-acetyltransferase deficiency
A coupled assay detecting defects in fibroblast isoleucine degradation distal to enoyl-CoA hydratase: application to 3-oxothiolase deficiency.
Acidosis
Erratum to: Novel ECHS1 mutation in an Emirati neonate with severe metabolic acidosis.
Acidosis
Novel ECHS1 mutation in an Emirati neonate with severe metabolic acidosis.
Acidosis
Reveal genes functionally associated with ACADS by a network study.
Acidosis, Lactic
An incidental finding in newborn screening leading to the diagnosis of a patient with ECHS1 mutations.
Acidosis, Lactic
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Acidosis, Lactic
ECHS1 Deficiency as a Cause of Severe Neonatal Lactic Acidosis.
Acidosis, Lactic
Two novel ECHS1 variants, affecting splicing and reducing enzyme activity, is associated with mitochondrial encephalopathy in infant: a case report.
acyl-coa oxidase deficiency
Distinction between peroxisomal bifunctional enzyme and acyl-CoA oxidase deficiencies.
Adrenoleukodystrophy
Clinical consequences of defects in peroxisomal beta-oxidation.
alpha-methylacyl-coa racemase deficiency
Clinical consequences of defects in peroxisomal beta-oxidation.
Atherosclerosis
Common dysregulated pathways in obese adipose tissue and atherosclerosis.
Brain Diseases
Deficiency of ECHS1 causes mitochondrial encephalopathy with cardiac involvement.
Brain Diseases
Dystonia-ataxia syndrome with permanent torsional nystagmus caused by ECHS1 deficiency.
Brain Diseases
Mitochondrial Encephalopathy and Transient 3-Methylglutaconic Aciduria in ECHS1 Deficiency: Long-Term Follow-Up.
Brain Diseases
Novel ECHS1 mutation in an Emirati neonate with severe metabolic acidosis.
Brain Diseases
Two novel ECHS1 variants, affecting splicing and reducing enzyme activity, is associated with mitochondrial encephalopathy in infant: a case report.
Brain Diseases, Metabolic
Phenotypic spectrum of short-chain enoyl-Coa hydratase-1 (ECHS1) deficiency.
Breast Neoplasms
MiR-548-3p functions as an anti-oncogenic regulator in breast cancer.
Breast Neoplasms
The role of enoyl-CoA hydratase short chain 1 and peroxiredoxin 3 in PP2-induced apoptosis in human breast cancer MCF-7 cells.
Carcinogenesis
Enoyl-coenzyme A hydratase short chain 1 silencing attenuates the proliferation of hepatocellular carcinoma by inhibiting epidermal growth factor signaling in vitro and in vivo.
Carcinogenesis
Increased EHHADH Expression Predicting Poor Survival of Osteosarcoma by Integrating Weighted Gene Coexpression Network Analysis and Experimental Validation.
Carcinoma
ECHS1 suppresses renal cell carcinoma development through inhibiting mTOR signaling activation.
Carcinoma
Peroxisomes in human colon carcinomas. A cytochemical and biochemical study.
Carcinoma
The role of enoyl-CoA hydratase short chain 1 and peroxiredoxin 3 in PP2-induced apoptosis in human breast cancer MCF-7 cells.
Carcinoma, Hepatocellular
Decreased expression of the peroxisomal bifunctional enzyme and carbonyl reductase in human hepatocellular carcinomas.
Carcinoma, Hepatocellular
ECHS1 acts as a novel HBsAg-binding protein enhancing apoptosis through the mitochondrial pathway in HepG2 cells.
Carcinoma, Hepatocellular
Enoyl-coenzyme A hydratase short chain 1 silencing attenuates the proliferation of hepatocellular carcinoma by inhibiting epidermal growth factor signaling in vitro and in vivo.
Carcinoma, Hepatocellular
Knockdown of ECHS1 protein expression inhibits hepatocellular carcinoma cell proliferation via suppression of Akt activity.
Carcinoma, Hepatocellular
Silencing ECHS1 attenuates the proliferation and induces the autophagy of hepatocellular carcinoma via impairing cell metabolism and activating AMPK.
Carcinoma, Hepatocellular
Small hepatitis B surface antigen interacts with and modulates enoyl-coenzyme A hydratase expression in hepatoma cells.
Carcinoma, Hepatocellular
trans-activation of PPARalpha and induction of PPARalpha target genes by perfluorooctane-based chemicals.
Carcinoma, Renal Cell
ECHS1 suppresses renal cell carcinoma development through inhibiting mTOR signaling activation.
Cardiomyopathies
Novel ECHS1 mutation in an Emirati neonate with severe metabolic acidosis.
Cardiomyopathies
Two novel ECHS1 variants, affecting splicing and reducing enzyme activity, is associated with mitochondrial encephalopathy in infant: a case report.
Cardiomyopathy, Dilated
"Omics" data integration and functional analyses link Enoyl-CoA hydratase, short chain 1 to drug refractory dilated cardiomyopathy.
Chorea
Paroxysmal exercise-induced dystonia within the phenotypic spectrum of ECHS1 deficiency.
Colonic Neoplasms
Developing antineoplastic agents that target peroxisomal enzymes: cytisine-linked isoflavonoids as inhibitors of hydroxysteroid 17-beta-dehydrogenase-4 (HSD17B4).
Colorectal Neoplasms
Attenuation of enoyl coenzyme A hydratase 1 expression in colorectal cancer cells using small interfering RNA inhibits cell proliferation and migration.
Cutis Laxa
An incidental finding in newborn screening leading to the diagnosis of a patient with ECHS1 mutations.
Cutis Laxa
Unique presentation of cutis laxa with Leigh-like syndrome due to ECHS1 deficiency.
Deafness
Two novel ECHS1 variants, affecting splicing and reducing enzyme activity, is associated with mitochondrial encephalopathy in infant: a case report.
Dehydration
Channeling of 3-hydroxy-4-trans-decenoyl coenzyme A on the bifunctional beta-oxidation enzyme from rat liver peroxisomes and on the large subunit of the fatty acid oxidation complex from Escherichia coli.
Dehydration
D-3-hydroxyacyl coenzyme A dehydratase from rat liver peroxisomes. Purification and characterization of a novel enzyme necessary for the epimerization of 3-hydroxyacyl-CoA thioesters.
Dehydration
Enoyl-coenzyme A hydratase-catalyzed exchange of the alpha-protons of coenzyme A thiol esters: a model for an enolized intermediate in the enzyme-catalyzed elimination?
Dehydration
Isotope effects on the crotonase reaction.
Dyskinesias
ECHS1 deficiency-associated paroxysmal exercise-induced dyskinesias: case presentation and initial benefit of intervention.
Dyskinesias
Paroxysmal Exercise-Induced Dyskinesia in Siblings due to ECHS1 Gene Mutation - First Indian Case Report.
Dystonia
An incidental finding in newborn screening leading to the diagnosis of a patient with ECHS1 mutations.
Dystonia
Paroxysmal and non-paroxysmal dystonia in 3 patients with biallelic ECHS1 variants: Expanding the neurological spectrum and therapeutic approaches.
Dystonia
Paroxysmal exercise-induced dystonia within the phenotypic spectrum of ECHS1 deficiency.
Dystonia
Two novel ECHS1 variants, affecting splicing and reducing enzyme activity, is associated with mitochondrial encephalopathy in infant: a case report.
Dystonic Disorders
Paroxysmal exercise-induced dystonia within the phenotypic spectrum of ECHS1 deficiency.
enoyl-coa hydratase deficiency
A lethal neonatal phenotype of mitochondrial short-chain enoyl-CoA hydratase-1 deficiency.
enoyl-coa hydratase deficiency
A novel HPLC-based method to diagnose peroxisomal D-bifunctional protein enoyl-CoA hydratase deficiency.
enoyl-coa hydratase deficiency
An incidental finding in newborn screening leading to the diagnosis of a patient with ECHS1 mutations.
enoyl-coa hydratase deficiency
Case report and novel treatment of an autosomal recessive Leigh syndrome caused by short-chain enoyl-CoA hydratase deficiency.
enoyl-coa hydratase deficiency
Clinical, 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 excretion.
enoyl-coa hydratase deficiency
Clinical, biochemical, and genetic features of four patients with short-chain enoyl-CoA hydratase (ECHS1) deficiency.
enoyl-coa hydratase deficiency
Deficiency of ECHS1 causes mitochondrial encephalopathy with cardiac involvement.
enoyl-coa hydratase deficiency
Dystonia-ataxia syndrome with permanent torsional nystagmus caused by ECHS1 deficiency.
enoyl-coa hydratase deficiency
ECHS1 Deficiency as a Cause of Severe Neonatal Lactic Acidosis.
enoyl-coa hydratase deficiency
ECHS1 Mutations Cause Combined Respiratory Chain Deficiency Resulting in Leigh Syndrome.
enoyl-coa hydratase deficiency
Enoyl-CoA hydratase deficiency: identification of a new type of D-bifunctional protein deficiency.
enoyl-coa hydratase deficiency
Extrapolation of Variant Phase in Mitochondrial Short-Chain Enoyl-CoA Hydratase (ECHS1) Deficiency.
enoyl-coa hydratase deficiency
Mitochondrial Encephalopathy and Transient 3-Methylglutaconic Aciduria in ECHS1 Deficiency: Long-Term Follow-Up.
enoyl-coa hydratase deficiency
Mitochondrial Fatty Acid Oxidation Disorders Associated with Short-Chain Enoyl-CoA Hydratase (ECHS1) Deficiency.
enoyl-coa hydratase deficiency
Novel ECHS1 mutation in an Emirati neonate with severe metabolic acidosis.
enoyl-coa hydratase deficiency
Novel ECHS1 mutations in Leigh syndrome identified by whole-exome sequencing in five Chinese families: case report.
enoyl-coa hydratase deficiency
Paroxysmal exercise-induced dystonia within the phenotypic spectrum of ECHS1 deficiency.
enoyl-coa hydratase deficiency
Peroxisomal bifunctional enzyme complex deficiency with associated retinal findings.
enoyl-coa hydratase deficiency
Peroxisomal bifunctional enzyme deficiency with associated retinal findings.
enoyl-coa hydratase deficiency
Peroxisomal bifunctional enzyme deficiency.
enoyl-coa hydratase deficiency
Peroxisomal bifunctional enzyme deficiency: serial neurophysiological examinations of a case.
enoyl-coa hydratase deficiency
Short-chain enoyl-CoA hydratase deficiency causes prominent ketoacidosis with normal plasma lactate levels: A case report.
enoyl-coa hydratase deficiency
Understanding the role of OXPHOS dysfunction in the pathogenesis of ECHS1 deficiency.
enoyl-coa hydratase deficiency
Unique presentation of cutis laxa with Leigh-like syndrome due to ECHS1 deficiency.
enoyl-coa hydratase deficiency
Urinary bile acids and peroxisomal bifunctional enzyme deficiency.
enoyl-coa hydratase deficiency
Valine-restricted diet for patients with ECHS1 deficiency: Divergent clinical outcomes in two Japanese siblings.
Epilepsy
Two novel ECHS1 variants, affecting splicing and reducing enzyme activity, is associated with mitochondrial encephalopathy in infant: a case report.
Glioblastoma
Suppression of virus replication via down-modulation of mitochondrial short chain enoyl-CoA hydratase in human glioblastoma cells.
Hepatic Encephalopathy
Reveal genes functionally associated with ACADS by a network study.
Hepatitis
ECHS1 acts as a novel HBsAg-binding protein enhancing apoptosis through the mitochondrial pathway in HepG2 cells.
Hepatitis B
Small hepatitis B surface antigen interacts with and modulates enoyl-coenzyme A hydratase expression in hepatoma cells.
Hepatomegaly
Amino acid and nucleotide sequences of human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase cDNA.
Hepatomegaly
Peroxisome proliferation and induction of peroxisomal enzymes in mouse and rat liver by dehydroepiandrosterone feeding.
Insulin Resistance
Enoyl coenzyme A hydratase 1 protects against high-fat-diet-induced hepatic steatosis and insulin resistance.
Insulin Resistance
Genetic Regulation of Enoyl-CoA Hydratase Domain-Containing 3 in Adipose Tissue Determines Insulin Sensitivity in African Americans and Europeans.
Ketosis
Reveal genes functionally associated with ACADS by a network study.
Ketosis
Short-chain enoyl-CoA hydratase deficiency causes prominent ketoacidosis with normal plasma lactate levels: A case report.
Leigh Disease
A novel compound heterozygous variant of ECHS1 identified in a Japanese patient with Leigh syndrome.
Leigh Disease
An incidental finding in newborn screening leading to the diagnosis of a patient with ECHS1 mutations.
Leigh Disease
Case report and novel treatment of an autosomal recessive Leigh syndrome caused by short-chain enoyl-CoA hydratase deficiency.
Leigh Disease
ECHS1 Mutations Cause Combined Respiratory Chain Deficiency Resulting in Leigh Syndrome.
Leigh Disease
ECHS1 mutations in Leigh disease: a new inborn error of metabolism affecting valine metabolism.
Leigh Disease
Novel ECHS1 mutations in Leigh syndrome identified by whole-exome sequencing in five Chinese families: case report.
Leigh Disease
Understanding the role of OXPHOS dysfunction in the pathogenesis of ECHS1 deficiency.
Leigh Disease
Valine-restricted diet for patients with ECHS1 deficiency: Divergent clinical outcomes in two Japanese siblings.
Leigh Disease
Whole-exome sequencing identifies novel ECHS1 mutations in Leigh syndrome.
Leprosy
Enoyl-coenzyme A hydratase and antigen 85B of Mycobacterium habana are specifically recognized by antibodies in sera from leprosy patients.
Liver Diseases
Functional proteomic analysis of nonalcoholic fatty liver disease in rat models: enoyl-coenzyme a hydratase down-regulation exacerbates hepatic steatosis.
Liver Neoplasms
Attenuation of enoyl coenzyme A hydratase 1 expression in colorectal cancer cells using small interfering RNA inhibits cell proliferation and migration.
Liver Neoplasms
Competing endogenous RNA screening based on long noncoding RNA-messenger RNA co-expression profile in Hepatitis B virus-associated hepatocarcinogenesis.
long-chain-3-hydroxyacyl-coa dehydrogenase deficiency
Mitochondrial trifunctional protein deficiency. Catalytic heterogeneity of the mutant enzyme in two patients.
Lymphatic Metastasis
Enoyl coenzyme A hydratase 1 is an important factor in the lymphatic metastasis of tumors.
Lymphatic Metastasis
Expression of enoyl coenzyme A hydratase, short chain, 1, in colorectal cancer and its association with clinicopathological characteristics.
Lymphoma
Signalling pathways identified in salivary glands from primary Sjögren's syndrome patients reveal enhanced adipose tissue development.
Metabolism, Inborn Errors
Short-chain enoyl-CoA hydratase deficiency causes prominent ketoacidosis with normal plasma lactate levels: A case report.
Mitochondrial Diseases
ECHS1 Mutations Cause Combined Respiratory Chain Deficiency Resulting in Leigh Syndrome.
Mitochondrial Diseases
Two novel ECHS1 variants, affecting splicing and reducing enzyme activity, is associated with mitochondrial encephalopathy in infant: a case report.
Muscle Hypotonia
Amino acid and nucleotide sequences of human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase cDNA.
Muscle Hypotonia
Novel ECHS1 mutation in an Emirati neonate with severe metabolic acidosis.
Muscle Hypotonia
Peroxisomal bifunctional enzyme deficiency with associated retinal findings.
Muscular Diseases
Reveal genes functionally associated with ACADS by a network study.
Neoplasm Metastasis
Expression of enoyl coenzyme A hydratase, short chain, 1, in colorectal cancer and its association with clinicopathological characteristics.
Neoplasms
Attenuation of enoyl coenzyme A hydratase short chain 1 expression in gastric cancer cells inhibits cell proliferation and migration in vitro.
Neoplasms
Attenuation of enoyl coenzyme A hydratase 1 expression in colorectal cancer cells using small interfering RNA inhibits cell proliferation and migration.
Neoplasms
Enoyl coenzyme A hydratase 1 is an important factor in the lymphatic metastasis of tumors.
Neoplasms
Enoyl-CoA hydratase-1 regulates mTOR signaling and apoptosis by sensing nutrients.
Neoplasms
Enoyl-coenzyme A hydratase in cancer.
Neoplasms
Enoyl-coenzyme A hydratase short chain 1 silencing attenuates the proliferation of hepatocellular carcinoma by inhibiting epidermal growth factor signaling in vitro and in vivo.
Neoplasms
Increased EHHADH Expression Predicting Poor Survival of Osteosarcoma by Integrating Weighted Gene Coexpression Network Analysis and Experimental Validation.
Neoplasms
Knockdown of ECHS1 protein expression inhibits hepatocellular carcinoma cell proliferation via suppression of Akt activity.
Neoplasms
Micropreparative immobilized pH gradient two-dimensional electrophoresis in combination with protein microsequencing for the analysis of human liver proteins.
Neoplasms
Silencing ECHS1 attenuates the proliferation and induces the autophagy of hepatocellular carcinoma via impairing cell metabolism and activating AMPK.
Non-alcoholic Fatty Liver Disease
Enoyl coenzyme A hydratase 1 alleviates nonalcoholic steatohepatitis in mice by suppressing hepatic ferroptosis.
Non-alcoholic Fatty Liver Disease
Functional proteomic analysis of nonalcoholic fatty liver disease in rat models: enoyl-coenzyme a hydratase down-regulation exacerbates hepatic steatosis.
Obesity
Enoyl coenzyme A hydratase 1 combats obesity and related metabolic disorders by promoting adipose tissue browning.
Optic Atrophy
Two novel ECHS1 variants, affecting splicing and reducing enzyme activity, is associated with mitochondrial encephalopathy in infant: a case report.
Protein Deficiency
D-bifunctional protein deficiency: a cause of neonatal onset seizures and hypotonia.
Protein Deficiency
Enoyl-CoA hydratase deficiency: identification of a new type of D-bifunctional protein deficiency.
pyruvate decarboxylase deficiency
Lethal neonatal case and review of primary short-chain enoyl-CoA hydratase (SCEH) deficiency associated with secondary lymphocyte pyruvate dehydrogenase complex (PDC) deficiency.
pyruvate dehydrogenase (acetyl-transferring) deficiency
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Pyruvate Dehydrogenase Complex Deficiency Disease
Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.
Reye Syndrome
Reveal genes functionally associated with ACADS by a network study.
Sarcoma
High-performance liquid chromatography/nano-electrospray ionization tandem mass spectrometry, two-dimensional difference in-gel electrophoresis and gene microarray identification of lymphatic metastasis-associated biomarkers.
Seizures
Peroxisomal bifunctional enzyme deficiency with associated retinal findings.
Starvation
Mevalonate governs interdependency of ergosterol and siderophore biosyntheses in the fungal pathogen Aspergillus fumigatus.
Starvation
Regulation of the pehA gene encoding the major polygalacturonase of Xanthomonas campestris by Clp and RpfF.
Stomach Neoplasms
Attenuation of enoyl coenzyme A hydratase short chain 1 expression in gastric cancer cells inhibits cell proliferation and migration in vitro.
Stomach Neoplasms
Differentially expressed genes PCCA, ECHS1, and HADH are potential prognostic biomarkers for gastric cancer.
Tuberculosis
A distinct MaoC-like enoyl-CoA hydratase architecture mediates cholesterol catabolism in Mycobacterium tuberculosis.
Tuberculosis
Cloning of an ORF with homology to Mycobacterium echA1, encoding the enoyl-CoA hydratase, in Rhodococcus fascians.
Tuberculosis
Multiparameter screening on SlipChip used for nanoliter protein crystallization combining free interface diffusion and microbatch methods.
Tuberculosis
Mycobacterium tuberculosis Exploits a Heterohexameric Enoyl-CoA Hydratase Retro-Aldolase Complex for Cholesterol Catabolism.
Tuberculosis
Post-translational Succinylation of Mycobacterium tuberculosis Enoyl-CoA Hydratase EchA19 Slows Catalytic Hydration of Cholesterol Catabolite 3-Oxo-chol-4,22-diene-24-oyl-CoA.
Urinary Bladder Neoplasms
Search for the tumor-related proteins of transition cell carcinoma in Taiwan by proteomic analysis.
Zellweger Syndrome
Amino acid and nucleotide sequences of human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase cDNA.
Zellweger Syndrome
Neuronal migration abnormality in peroxisomal bifunctional enzyme defect.
Zellweger Syndrome
Peroxisomal beta-oxidation enzyme proteins in the Zellweger syndrome.
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4.2.1.17 enoyl-CoA hydratase
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The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). 
