Gene name: ECHS1

Uniprot entry:

P30084

Protein names:

Enoyl-CoA hydratase, mitochondrial (mECH) (mECH1) (EC 4.2.1.17) (EC 5.3.3.8) (Enoyl-CoA hydratase 1) (ECHS1) (Short-chain enoyl-CoA hydratase) (SCEH)

Protein sequence:

1_MAALR 6_ VLLSC 11_ VRGPL 16_ RPPVR 21_ CPAWR 26_ PFASG 31_ ANFEY 36_ IIAEK 41_ RGKNN 46_ TVGLI 51_ QLNRP 56_ KALNA 61_ LCDGL 66_ IDELN 71_ QALKT 76_ FEEDP 81_ AVGAI 86_ VLTGG 91_ DKAFA 96_ AGADI 101_ KEMQN 106_ LSFQD 111_ CYSSK 116_ FLKHW 121_ DHLTQ 126_ VKKPV 131_ IAAVN 136_ GYAFG 141_ GGCEL 146_ AMMCD 151_ IIYAG 156_ EKAQF 161_ AQPEI 166_ LIGTI 171_ PGAGG 176_ TQRLT 181_ RAVGK 186_ SLAME 191_ MVLTG 196_ DRISA 201_ QDAKQ 206_ AGLVS 211_ KICPV 216_ ETLVE 221_ EAIQC 226_ AEKIA 231_ SNSKI 236_ VVAMA 241_ KESVN 246_ AAFEM 251_ TLTEG 256_ SKLEK 261_ KLFYS 266_ TFATD 271_ DRKEG 276_ MTAFV 281_EKRKA

Protein annotations

Protein functions:

1: 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)