Search Results

Overview

Uniprot IDP30084
Protein NameEnoyl-CoA hydratase, mitochondrial
Gene NameECHS1
OrganismHomo sapiens

Kla Sites from experimental identification

Position Flanking peptide
115 FQDCYSSKFLKHWDH
118 CYSSKFLKHWDHLTQ
127 WDHLTQVKKPVIAAV
185 RLTRAVGKSLAMEMV
204 RISAQDAKQAGLVSK
234 EKIASNSKIVVAMAK
261 EGSKLEKKLFYSTFA
273 TFATDDRKEGMTAFV
282 GMTAFVEKRKANFKD
43 IIAEKRGKNNTVGLI
56 LIQLNRPKALNALCD

Function

Converts unsaturated trans-2-enoyl-CoA species ((2E)-enoyl-CoA) to the corresponding (3S)-3hydroxyacyl-CoA species through addition of a water molecule to the double bond (PubMed:25125611, PubMed:26251176). Catalyzes the hydration of medium- and short-chained fatty enoyl-CoA thioesters from 4 carbons long (C4) up to C16 (PubMed:26251176). Has high substrate specificity for crotonyl-CoA ((2E)-butenoyl-CoA) and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA (3-methyl-(2E)-butenoyl-CoA) and methacrylyl-CoA ((2E)-2-methylpropenoyl-CoA) (PubMed:26251176). Can bind tiglyl-CoA (2-methylcrotonoyl-CoA), but hydrates only a small amount of this substrate (PubMed:26251176). Plays a key role in the beta-oxidation spiral of short- and medium-chain fatty acid oxidation (PubMed:25125611, PubMed:26251176). At a lower rate than the hydratase reaction, catalyzes the isomerase reaction of trans-3-enoyl-CoA species (such as (3E)-hexenoyl-CoA) to trans-2-enoyl-CoA species (such as (2E)-hexenoyl-CoA), which are subsequently hydrated to 3(S)-3-hydroxyacyl-CoA species (such as (3S)-hydroxyhexanoyl-CoA) (By similarity)

Protein Sequence

10 MAALRVLLSC 20 VRGPLRPPVR 30 CPAWRPFASG 40 ANFEYIIAEK 50 RGKNNTVGLI 60 QLNRPKALNA 70 LCDGLIDELN 80 QALKTFEEDP 90 AVGAIVLTGG 100 DKAFAAGADI 110 KEMQNLSFQD 120 CYSSKFLKHW 130 DHLTQVKKPV 140 IAAVNGYAFG 150 GGCELAMMCD 160 IIYAGEKAQF 170 AQPEILIGTI 180 PGAGGTQRLT 190 RAVGKSLAME 200 MVLTGDRISA 210 QDAKQAGLVS 220 KICPVETLVE 230 EAIQCAEKIA 240 SNSKIVVAMA 250 KESVNAAFEM 260 TLTEGSKLEK 270 KLFYSTFATD 280 DRKEGMTAFV 290 EKRKANFKDQ

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0005739 mitochondrion
Molecular Function GO:0120092 (2E)-butenoyl-CoA hydratase activity
Molecular Function GO:0043956 3-hydroxypropionyl-CoA dehydratase activity
Molecular Function GO:0004165 delta(3)-delta(2)-enoyl-CoA isomerase activity
Molecular Function GO:0004300 enoyl-CoA hydratase activity
Biological Process GO:0009083 branched-chain amino acid catabolic process
Biological Process GO:0006635 fatty acid beta-oxidation
Biological Process GO:0006574 L-valine catabolic process

Reference

[1] Yang Z, Yan C, Ma J, Peng P, Ren X et al.. Lactylome analysis suggests lactylation-dependent mechanisms of metabolic adaptation in hepatocellular carcinoma.. Nat Metab 5(1):61-79. 2023 Jan. PMID: 36593272.

[2] Hong H, Chen X, Wang H, Gu X, Yuan Y et al.. Global profiling of protein lysine lactylation and potential target modified protein analysis in hepatocellular carcinoma.. Proteomics 23(9):e2200432. 2023 May. PMID: 36625413.

[3] Shi CM, Wang QC, Li XL, Yang YH, Tang XY et al.. Global Profiling of Protein Lactylation in Human Hippocampi.. Proteomics Clin Appl 19(2):e202400061. 2025 Mar. PMID: 39610256.

[4] Wu Q, Li Z, Gong T, Zheng X, Zhou X et al.. Porphyromonas gingivalis infection induces lysine lactylation reprogramming in human umbilical vein endothelial cells.. Front Cell Infect Microbiol 16:1706727. 2026. PMID: 41696360.