AiPD: Autoinhibited Protein Database

Gene name	DBT
Protein name	Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
Names	Branched-chain alpha-keto acid dehydrogenase complex component E2, BCKAD-E2, BCKADE2, Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, Dihydrolipoamide branched chain transacylase, Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase
Species	Bos taurus (Bovine)
KEGG Pathway	bta:280759
EC number	2.3.1.168: Transferring groups other than amino-acyl groups
Protein Class

The autoinhibited protein was predicted that may have potential autoinhibitory elements via cis-regPred.

20-91 (Predicted disordered autoinhibitory region) PR

20-91 (Predicted disordered autoinhibitory region)

cis-regPred

No accessory elements

Entry ID	Method	Resolution	Chain	Position	Source
2IHW	X-ray	270 A	A/B/C/D/E/F/G/H	223-482	PDB
2II3	X-ray	217 A	A/B/C/D/E/F/G/H	223-482	PDB
2II4	X-ray	259 A	A/B/C/D/E/F/G/H	223-482	PDB
2II5	X-ray	250 A	A/B/C/D/E/F/G/H	223-482	PDB
AF-P11181-F1	Predicted				AlphaFoldDB

Variant ID(s)	Position	Change	Diseaes Association	Provenance
rs473706473	7	L>V	No	EVA
rs459345826	11	S>R	No	EVA
rs474563711	11	S>T	No	EVA
rs463415269	17	L>P	No	EVA
rs463415269	17	L>R	No	EVA
rs211358391	24	Q>E	No	EVA
rs467577507	71	D>H	No	EVA
rs455667313	81	V>I	No	EVA
rs474549457	86	V>A	No	EVA
rs456941513	87	K>I	No	EVA
rs475664812	87	K>N	No	EVA
rs439361384	88	E>*	No	EVA
rs482333958	145	D>E	No	EVA
rs459217232	148	E>*	No	EVA
rs450904766	179	R>H	No	EVA
rs477886438	182	M>I	No	EVA
rs470321225	217	L>F	No	EVA
rs437293364	221	P>Q	No	EVA
rs452483966	239	I>L	No	EVA
rs464551291	245	P>S	No	EVA
rs478207805	247	F>L	No	EVA
rs434928046	249	G>D	No	EVA
rs475095933	250	K>I	No	EVA
rs476166439	258	G>S	No	EVA
rs518211012	267	M>I	No	EVA
rs520064887	270	A>V	No	EVA
rs3423155747	314	A>V	No	EVA
rs383768015	335	N>K	No	EVA
rs465383537	340	A>S	No	EVA
rs432323072	352	Q>P	No	EVA
rs454253077	358	N>I	No	EVA
rs440957332	380	L>R	No	EVA
rs436630311	395	T>M	No	EVA
rs442452339	412	V>E	No	EVA
rs461168004	413	I>M	No	EVA
rs482858411	414	L>F	No	EVA
rs450030989	415	P>R	No	EVA
rs465246451	418	V>G	No	EVA
rs447681908	419	A>G	No	EVA
rs477442292	419	A>S	No	EVA
rs436501750	423	L>V	No	EVA
rs454912621	427	K>M	No	EVA
rs479718825	428	A>D	No	EVA
rs440451014	430	P>S	No	EVA
rs470076849	431	R>Q	No	EVA
rs480823407	433	N>K	No	EVA
rs525392924	434	E>Q	No	EVA
rs1116750703	443	I>V	No	EVA

Type	Name	Position	InterPro Accession
domain	Biotin/lipoyl attachment	64 - 139	IPR000089
domain	2-oxoacid dehydrogenase acyltransferase, catalytic domain	250 - 479	IPR001078
binding_site	2-oxo acid dehydrogenase, lipoyl-binding site	89 - 118	IPR003016
domain	Peripheral subunit-binding domain	172 - 209	IPR004167

		Description
EC Number	2.3.1.168	Transferring groups other than amino-acyl groups
Subcellular Localization	Mitochondrion matrix
PANTHER Family
PANTHER Subfamily
PANTHER Protein Class
PANTHER Pathway Category	No pathway information available

Name	Definition
cytoplasm	The contents of a cell excluding the plasma membrane and nucleus, but including other subcellular structures.
cytosol	The part of the cytoplasm that does not contain organelles but which does contain other particulate matter, such as protein complexes.
microtubule cytoskeleton	The part of the cytoskeleton (the internal framework of a cell) composed of microtubules and associated proteins.
mitochondrial alpha-ketoglutarate dehydrogenase complex	Mitochondrial complex that possesses alpha-ketoglutarate dehydrogenase activity.
mitochondrial nucleoid	The region of a mitochondrion to which the DNA is confined.
mitochondrion	A semiautonomous, self replicating organelle that occurs in varying numbers, shapes, and sizes in the cytoplasm of virtually all eukaryotic cells. It is notably the site of tissue respiration.

Name	Definition
acetyltransferase activity	Catalysis of the transfer of an acetyl group to an acceptor molecule.
dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity	Catalysis of the reaction: 2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine = CoA + enzyme N6-(S-dihydrolipoyl)lysine.
lipoic acid binding	Binding to lipoic acid, 1,2-dithiolane-3-pentanoic acid.
ubiquitin protein ligase binding	Binding to a ubiquitin protein ligase enzyme, any of the E3 proteins.

Name	Definition
branched-chain amino acid catabolic process	The chemical reactions and pathways resulting in the breakdown of amino acids containing a branched carbon skeleton, comprising isoleucine, leucine and valine.

UniProt AC	Gene Name	Protein Name	Species	Evidence Code
P11182	DBT	Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial	Homo sapiens (Human)	PR
P53395	Dbt	Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial	Mus musculus (Mouse)	PR
Q23571	dbt-1	Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial	Caenorhabditis elegans	PR
Q9M7Z1	BCE2	Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial	Arabidopsis thaliana (Mouse-ear cress)	PR
Q5M729	At1g54220	Dihydrolipoyllysine-residue acetyltransferase component 3 of pyruvate dehydrogenase complex, mitochondrial	Arabidopsis thaliana (Mouse-ear cress)	PR
Q8RWN9	At3g13930	Dihydrolipoyllysine-residue acetyltransferase component 2 of pyruvate dehydrogenase complex, mitochondrial	Arabidopsis thaliana (Mouse-ear cress)	PR