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ECHS1 Matched Antibody Pair (361) (APMAB-361LY)

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Specifications

ApplIcation
Sandwich ELISA
Specificity
Human
Capture Antibody
Rabbit anti-ECHS1 polyclonal antibody, 100 ug
Detection Antibody
Anti-ECHS1 Mouse monoclonal, IgG1 antibody, 20 ug
Dilutions
10 ng/ml-100 ng/ml
Format
Liquid
Storage
Aliquot and store at -20°Cor -80°C. Avoid freeze-thaw cycles.
Alternative Names
SCEH
Entrez Gene ID
More Infomation

Pata, S., Flores-Rojas, K., Gil, A., López-Laso, E., Marti-Sánchez, L., Baide-Mairena, H., ... & Gil-Campos, M. (2022). 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. Orphanet Journal of Rare Diseases, 17(1), 1-10.

Muntean, C., Tripon, F., Bogliș, A., & Bănescu, C. (2022). Pathogenic Biallelic Mutations in ECHS1 in a Case with Short-Chain Enoyl-CoA Hydratase (SCEH) Deficiency-Case Report and Literature Review. International Journal of Environmental Research and Public Health, 19(4), 2088.

Cai, K., Wang, F., Lu, J. Q., Shen, A. N., Zhao, S. M., Zang, W. D., ... & Zhao, J. Y. (2022). Nicotinamide mononucleotide alleviates cardiomyopathy phenotypes caused by short-chain enoyl-CoA hydratase 1 deficiency. Basic to Translational Science, 7(4), 348-362.

Yang, H., & Yu, D. (2020). Clinical, biochemical and metabolic characterization of patients with short-chain enoyl-CoA hydratase (ECHS1) deficiency: two case reports and the review of the literature. BMC pediatrics, 20(1), 1-10.

Masnada, S., Parazzini, C., Bini, P., Barbarini, M., Alberti, L., Valente, M., ... & Tonduti, D. (2020). Phenotypic spectrum of short-chain enoyl-Coa hydratase-1 (ECHS1) deficiency. European Journal of Paediatric Neurology, 28, 151-158.

Fitzsimons, P. E., Alston, C. L., Bonnen, P. E., Hughes, J., Crushell, E., Geraghty, M. T., ... & Mayne, P. D. (2018). Clinical, biochemical, and genetic features of four patients with short‐chain enoyl‐CoA hydratase (ECHS1) deficiency. American Journal of Medical Genetics Part A, 176(5), 1115-1127.

Carlston, C. M., Ferdinandusse, S., Hobert, J. A., Mao, R., & Longo, N. (2018). Extrapolation of variant phase in mitochondrial short-chain Enoyl-CoA Hydratase (ECHS1) deficiency. In JIMD Reports, Volume 43 (pp. 103-109). Springer, Berlin, Heidelberg.

Sharpe, A. J., & McKenzie, M. (2018). Mitochondrial fatty acid oxidation disorders associated with short-chain enoyl-CoA hydratase (ECHS1) deficiency. Cells, 7(6), 46.

Campbell, N. V., Weitzenkamp, D. A., Campbell, I. L., Schmidt, R. F., Hicks, C., Morgan, M. J., ... & Tentler, J. J. (2018). “Omics” data integration and functional analyses link Enoyl-CoA hydratase, short chain 1 to drug refractory dilated cardiomyopathy. BMC Medical Genomics, 11(1), 1-30.

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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