SMART: Pfam domain ECH

The domain within your query sequence starts at position 148 and ends at position 389; the E-value for the ECH_2 domain shown below is 6e-21.

ITKITFNRPTKKNAISFQMYRDIILALKNASTDNTVMAVFTGTGDYYCSGNDLTNFTSAT
GGIEEAASNGAVLLRDFVNSFIDFPKPLVAVVNGPAVGISVTLLGLFDAVFASDRATFHT
PFSQLGQSPEACSSYTFPKMMGSAKAAEMLLFGKKLTAREAWAQGLVTEVFPESTFETEV
WTRLKTYAKLPPNAMRISKELIRKNEKEKLYAVNAEECTTLQARWLSEECMNAIMSFVSR
KP

ECH_2

PFAM accession number:	PF16113
Interpro abstract (IPR032259):	The enoyl-CoA hydratase/isomerase family contains a diverse set of enzymes including: enoyl-CoA hydratase, napthoate synthase, carnitate racemase, 3-hydroxybutyryl-CoA dehydratase and dodecanoyl-CoA delta-isomerase. This entry represents a subset of the enoyl-CoA hydratase/isomerase family. Proteins in this entry includes 3-hydroxyisobutyryl-CoA hydrolases (HIBYL-CoA-H) from eukaryotes and their homologues from bacteria. Human HIBYL-CoA-H is a mitochondrial enzyme that catalyses the fifth step in the valine catabolic pathway in eukaryotes, namely the conversion of 3-hydroxyisobutyryl-CoA to free 3-hydroxyisobutyrate [ (PUBMED:8824301) (PUBMED:21104317) ]. It also hydrolyses 3-hydroxypropionyl-CoA, giving it a dual role in a secondary pathway of propionate metabolism [ (PUBMED:26163321) ]. Deficiency of this enzyme is associated with Leigh-like disease [ (PUBMED:25125611) (PUBMED:24299452) ].
GO function:	3-hydroxyisobutyryl-CoA hydrolase activity (GO:0003860)

PFAM accession number:

PF16113

Interpro abstract (IPR032259):

The enoyl-CoA hydratase/isomerase family contains a diverse set of enzymes including: enoyl-CoA hydratase, napthoate synthase, carnitate racemase, 3-hydroxybutyryl-CoA dehydratase and dodecanoyl-CoA delta-isomerase. This entry represents a subset of the enoyl-CoA hydratase/isomerase family. Proteins in this entry includes 3-hydroxyisobutyryl-CoA hydrolases (HIBYL-CoA-H) from eukaryotes and their homologues from bacteria.

Human HIBYL-CoA-H is a mitochondrial enzyme that catalyses the fifth step in the valine catabolic pathway in eukaryotes, namely the conversion of 3-hydroxyisobutyryl-CoA to free 3-hydroxyisobutyrate [ (PUBMED:8824301) (PUBMED:21104317) ]. It also hydrolyses 3-hydroxypropionyl-CoA, giving it a dual role in a secondary pathway of propionate metabolism [ (PUBMED:26163321) ]. Deficiency of this enzyme is associated with Leigh-like disease [ (PUBMED:25125611) (PUBMED:24299452) ].

GO function:

3-hydroxyisobutyryl-CoA hydrolase activity (GO:0003860)

This is a PFAM domain. For full annotation and more information, please see the PFAM entry ECH_2