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Anti-ECHS1 Antibody
Polyclonal Antibody against HUMAN ECHS1
0.2 mg/ml
Immunohistochemical staining of human gastrointestinal, heart muscle, kidney and liver using Anti-ECHS1 antibody HPA022476 (A) shows similar protein distribution across tissues to independent antibody HPA021995 (B).
The Anti‑ECHS1 antibody (HPA022476) is a highly specific, affinity‑purified rabbit polyclonal antibody targeting ECHS1—enoyl‑CoA hydratase, short chain 1—a mitochondrial matrix enzyme central to fatty‑acid β‑oxidation and branched‑chain amino‑acid metabolism. Validated for immunohistochemistry (IHC), immunocytochemistry–immunofluorescence (ICC‑IF), and Western blot (WB), this antibody shows robust mitochondrial localization by IF and the expected granular cytoplasmic staining in tissues with high mitochondrial content (e.g., hepatocytes, cardiomyocytes, renal tubules, and intestinal epithelium). Enhanced validation includes independent‑antibody concordance versus HPA021995, strengthening specificity and reproducibility across applications. Verified species reactivity includes human, mouse, and rat.
Significance in Research: ECHS1 catalyzes a key hydration step in mitochondrial fatty‑acid β‑oxidation, converting trans‑2‑enoyl‑CoA intermediates to 3‑hydroxyacyl‑CoAs, and also participates in branched‑chain amino‑acid (valine/isoleucine) catabolism. Through these pathways, ECHS1 supports cellular energy homeostasis; perturbations can lead to secondary OXPHOS dysfunction.
Clinically, ECHS1 deficiency (ECHS1D) is linked to severe mitochondrial encephalopathies, often with Leigh‑like presentations, lactic acidosis, and movement disorders. Structural and biochemical studies have illuminated substrate‑recognition mechanisms and how disease‑associated mutations disrupt activity, underlining ECHS1 as a pivotal node for metabolism‑focused disease modeling and therapeutic exploration.
Alternative antibodies
Corresponding antigens
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From our facilities in Stockholm, Sweden we develop, manufacture and distribute highly advanced reagents to the Life Sciences community worldwide.
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Learn how we validate our antibodies, how we secure their reproducibility, and why we apply enhanced validation. Our antibodies are validated in IHC, ICC-IF, and WB.