UniProt Accession

 ECHM_HUMAN; P30084; O00739; Q5VWY1; Q96H54 

Genbank Protein ID

 D13900; X98126; X98127; X98128; X98129; BT007123; AL360181; BC008906 

Genbank Nucleotide ID

 BAA03001.1; CAA66808.1; CAA66808.1; CAA66808.1; CAA66808.1; AAP35787.1; CAH70286.1; AAH08906.1 

Protein Name

 Enoyl-CoA hydratase, mitochondrial 

Protein Synonyms/Alias

 Enoyl-CoA hydratase 1; Short-chain enoyl-CoA hydratase; SCEH 

Gene Name

 ECHS1 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
115	FQDCYSSKFLKHWDH	acetylation	[1]
118	CYSSKFLKHWDHLTQ	acetylation	[1, 2, 3]
185	RLTRAVGKSLAMEMV	ubiquitination	[4]
204	RISAQDAKQAGLVSK	acetylation	[1]
204	RISAQDAKQAGLVSK	ubiquitination	[4, 5, 6, 7, 8]
261	EGSKLEKKLFYSTFA	ubiquitination	[5, 8, 9]

Reference

 [1] Proteomic investigations of lysine acetylation identify diverse substrates of mitochondrial deacetylase sirt3.
 Sol EM, Wagner SA, Weinert BT, Kumar A, Kim HS, Deng CX, Choudhary C.
 PLoS One. 2012;7(12):e50545. [PMID: 23236377]
 [2] Lysine acetylation targets protein complexes and co-regulates major cellular functions.
 Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M.
 Science. 2009 Aug 14;325(5942):834-40. [PMID: 19608861]
 [3] Integrated proteomic analysis of post-translational modifications by serial enrichment.
 Mertins P, Qiao JW, Patel J, Udeshi ND, Clauser KR, Mani DR, Burgess MW, Gillette MA, Jaffe JD, Carr SA.
 Nat Methods. 2013 Jul;10(7):634-7. [PMID: 23749302]
 [4] Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization.
 Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, Harper JW.
 Nature. 2013 Apr 18;496(7445):372-6. [PMID: 23503661]
 [5] A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles.
 Wagner SA, Beli P, Weinert BT, Nielsen ML, Cox J, Mann M, Choudhary C.
 Mol Cell Proteomics. 2011 Oct;10(10):M111.013284. [PMID: 21890473]
 [6] Systematic and quantitative assessment of the ubiquitin-modified proteome.
 Kim W, Bennett EJ, Huttlin EL, Guo A, Li J, Possemato A, Sowa ME, Rad R, Rush J, Comb MJ, Harper JW, Gygi SP.
 Mol Cell. 2011 Oct 21;44(2):325-40. [PMID: 21906983]
 [7] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961]
 [8] hCKSAAP_UbSite: improved prediction of human ubiquitination sites by exploiting amino acid pattern and properties.
 Chen Z, Zhou Y, Song J, Zhang Z.
 Biochim Biophys Acta. 2013 Aug;1834(8):1461-7. [PMID: 23603789]
 [9] Systems-wide analysis of ubiquitylation dynamics reveals a key role for PAF15 ubiquitylation in DNA-damage bypass.
 Povlsen LK, Beli P, Wagner SA, Poulsen SL, Sylvestersen KB, Poulsen JW, Nielsen ML, Bekker-Jensen S, Mailand N, Choudhary C.
 Nat Cell Biol. 2012 Oct;14(10):1089-98. [PMID: 23000965] 

Functional Description

 Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate. 

Sequence Annotation

 REGION 98 101 Substrate binding.
 BINDING 141 141 Substrate; via amide nitrogen.
 MOD_RES 101 101 N6-acetyllysine (By similarity).
 MOD_RES 118 118 N6-acetyllysine.  

Keyword

 3D-structure; Acetylation; Complete proteome; Direct protein sequencing; Fatty acid metabolism; Lipid metabolism; Lyase; Mitochondrion; Polymorphism; Reference proteome; Transit peptide. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 290 AA 

Protein Sequence

Gene Ontology

 GO:0005759; C:mitochondrial matrix; TAS:Reactome.
 GO:0004300; F:enoyl-CoA hydratase activity; TAS:Reactome.
 GO:0006635; P:fatty acid beta-oxidation; TAS:Reactome. 

Interpro

 IPR001753; Crotonase_core_superfam.
 IPR018376; Enoyl-CoA_hyd/isom_CS. 

Pfam

 PF00378; ECH 

SMART

  

PROSITE

 PS00166; ENOYL_COA_HYDRATASE 

PRINTS