Catalog No.
YHD87001
Expression system
E. coli
Species
Homo sapiens (Human)
Protein length
Ala28-Gln290
Predicted molecular weight
30.65 kDa
Nature
Recombinant
Endotoxin level
Please contact with the lab for this information.
Purity
>90% as determined by SDS-PAGE.
Accession
P30084
Applications
ELISA, Immunogen, SDS-PAGE, WB, Bioactivity testing in progress
Form
Lyophilized
Storage buffer
Lyophilized from a solution in PBS pH 7.4, 1mM EDTA, 4% Trehalose, 1% Mannitol.
Reconstitution
Reconstitute in sterile water for a stock solution.
Shipping
In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
Alternative Names
SCEH, ECHS1, Enoyl-CoA hydratase 1, Enoyl-CoA hydratase, mitochondrial, Short-chain enoyl-CoA hydratase
Deficiency in the conserved ECHS1 gene causes Leigh syndrome by impairing mitochondrial respiration efficiency and suppressing ADRB2-PKA signaling., PMID:40446940
Acetylation-induced degradation of ECHS1 enhances BCAA accumulation and proliferation in KRAS-mutant colorectal cancer., PMID:40437561
Comprehensive analysis of chronic hepatitis B concurrent with non-alcoholic fatty liver disease: a proteomics report based on clinical liver samples., PMID:40361009
Time-resolved mitochondrial screen identifies regulatory components of oxidative metabolism., PMID:40301572
Towards a kingdom of reproductive life - the core sperm proteome., PMID:40298292
Structural and biochemical mechanism of short-chain enoyl-CoA hydratase (ECHS1) substrate recognition., PMID:40240482
Expanding the Clinical and Genetic Spectrum of Mitochondrial Short-Chain Enoyl-CoA Hydratase 1 Deficiency: Insights From Two Unrelated Chinese Families., PMID:40192239
ECHS1 as a Lipid Metabolism Biomarker for Pediatric Focal Segmental Glomerulosclerosis., PMID:40063869
Ectopic protein lysine methacrylation contributes to defects caused by loss of HIBCH or ECHS1., PMID:40056416
ECHS1-NOX4 interaction suppresses rotenone-induced dopaminergic neurotoxicity through inhibition of mitochondrial ROS production., PMID:40032032
Valine Restriction Extends Survival in a Drosophila Model of Short-Chain Enoyl-CoA Hydratase 1 (ECHS1) Deficiency., PMID:39727068
Deciphering the role of tryptophan metabolism-associated genes ECHS1 and ALDH2 in gastric cancer: implications for tumor immunity and personalized therapy., PMID:39328412
Hypoxia-induced downregulation of PGK1 crotonylation promotes tumorigenesis by coordinating glycolysis and the TCA cycle., PMID:39134530
Valine and Inflammation Drive Epilepsy in a Mouse Model of ECHS1 Deficiency., PMID:38915588
Further delineation of short-chain enoyl-CoA hydratase deficiency in the Pacific population., PMID:38820906
Effects of Fat and Carnitine on the Expression of Carnitine Acetyltransferase and Enoyl-CoA Hydratase Short-Chain 1 in the Liver of Juvenile GIFT (Oreochromis niloticus)., PMID:38674414
Phytol and α-Bisabolol Synergy Induces Autophagy and Apoptosis in A549 Cells and Additional Molecular Insights through Comprehensive Proteome Analysis via Nano LC-MS/MS., PMID:38415491
Molecular and in silico investigation of a novel ECHS1 gene mutation in a consanguine family with short-chain enoyl-CoA hydratase deficiency and Mt-DNA depletion: effect on trimer assembly and catalytic activity., PMID:38363494
Expression and clinical significance of ECHS1 in gastric cancer., PMID:38169583
Transcriptome analysis reveal the effect of freshwater sediments containing 2,3,7,8-tetrachlorodibenzo-p-dioxin on the Macrobrachium rosenbergii hepatopancreas, intestine, and muscle., PMID:38110107
Acyl-CoA dehydrogenase substrate promiscuity: Challenges and opportunities for development of substrate reduction therapy in disorders of valine and isoleucine metabolism., PMID:37309295
Lysine catabolism reprograms tumour immunity through histone crotonylation., PMID:37198486
Coenzyme A binding sites induce proximal acylation across protein families., PMID:36977698
Dehydroepiandrosterone alleviates oleic acid-induced lipid metabolism disorders through activation of AMPK-mTOR signal pathway in primary chicken hepatocytes., PMID:36565630
Movement disorders in valine métabolism diseases caused by HIBCH and ECHS1 deficiencies., PMID:36200804
Exercise improves choroid plexus epithelial cells metabolism to prevent glial cell-associated neurodegeneration., PMID:36188600
Clinical improvements after treatment with a low-valine and low-fat diet in a pediatric patient with enoyl-CoA hydratase, short chain 1 (ECHS1) deficiency., PMID:36064416
Melatonin Ameliorates Cisplatin-Induced Renal Tubular Epithelial Cell Damage through PPARα/FAO Regulation., PMID:36006825
Loss of mitochondrial fatty acid β-oxidation protein short-chain Enoyl-CoA hydratase disrupts oxidative phosphorylation protein complex stability and function., PMID:35962613
ECHS1 deficiency and its biochemical and clinical phenotype., PMID:35856138
Betaine Supplementation Causes an Increase in Fatty Acid Oxidation and Carbohydrate Metabolism in Livers of Mice Fed a High-Fat Diet: A Proteomic Analysis., PMID:35327303
Pathogenic Biallelic Mutations in ECHS1 in a Case with Short-Chain Enoyl-CoA Hydratase (SCEH) Deficiency-Case Report and Literature Review., PMID:35206276
A Case of ECHS1 Deficiency with Severe Encephalopathy and Status Epilepticus after a Propofol Sedation: Case Report., PMID:35098523
Leigh Syndrome: A Study of 209 Patients at the Beijing Children's Hospital., PMID:35094435
SOX2 mediates metabolic reprogramming of prostate cancer cells., PMID:35067686
Whole-exome sequencing identified novel variants in three Chinese Leigh syndrome pedigrees., PMID:35014173
Valine metabolites analysis in ECHS1 deficiency., PMID:34667719
ECHS1, an interacting protein of LASP1, induces sphingolipid-metabolism imbalance to promote colorectal cancer progression by regulating ceramide glycosylation., PMID:34615856
Remodelin, a N-acetyltransferase 10 (NAT10) inhibitor, alters mitochondrial lipid metabolism in cancer cells., PMID:34605570
Imatinib protects against human beta-cell death via inhibition of mitochondrial respiration and activation of AMPK., PMID:34569605
HSP60 Regulates Lipid Metabolism in Human Ovarian Cancer., PMID:34557266
Thioesterase induction by 2,3,7,8-tetrachlorodibenzo-p-dioxin results in a futile cycle that inhibits hepatic β-oxidation., PMID:34344994
Differentially expressed genes PCCA, ECHS1, and HADH are potential prognostic biomarkers for gastric cancer., PMID:33881965
Cajanolactone A, a stilbenoid from Cajanus cajan, inhibits energy intake and lipid synthesis/storage, and promotes energy expenditure in ovariectomized mice., PMID:33744755
Valine-restricted diet for patients with ECHS1 deficiency: Divergent clinical outcomes in two Japanese siblings., PMID:33139125
ECHS1 disease in two unrelated families of Samoan descent: Common variant - rare disorder., PMID:33112498
Inhibitory mechanism of 6-Pentyl-2H-pyran-2-one secreted by Trichoderma atroviride T2 against Cylindrocarpon destructans., PMID:32980051
Paroxysmal and non-paroxysmal dystonia in 3 patients with biallelic ECHS1 variants: Expanding the neurological spectrum and therapeutic approaches., PMID:32858208
Zearalenone disrupts the placental function of rats: A possible mechanism causing intrauterine growth restriction., PMID:32858132
For research use only. Not for human or drug use.
