BAAT

Functionⁱ

Involved in bile acid metabolism. In liver hepatocytes catalyzes the second step in the conjugation of C24 bile acids (choloneates) to glycine and taurine before excretion into bile canaliculi. The major components of bile are cholic acid and chenodeoxycholic acid. In a first step the bile acids are converted to an acyl-CoA thioester, either in peroxisomes (primary bile acids deriving from the cholesterol pathway), or cytoplasmic at the endoplasmic reticulum (secondary bile acids). May catalyze the conjugation of primary or secondary bile acids, or both. The conjugation increases the detergent properties of bile acids in the intestine, which facilitates lipid and fat-soluble vitamin absorption. In turn, bile acids are deconjugated by bacteria in the intestine and are recycled back to the liver for reconjugation (secondary bile acids). May also act as an acyl-CoA thioesterase that regulates intracellular levels of free fatty acids. In vitro, catalyzes the hydrolysis of long- and very long-chain saturated acyl-CoAs to the free fatty acid and coenzyme A (CoASH), and conjugates glycine to these acyl-CoAs.2 Publications

Miscellaneous

In human, more than 95% of the biliary bile acids are N-acyl amidates with glycine and taurine. In other mammalian species large differences are observed in the relative amounts of taurine- and glycine-conjugated bile acids formed in bile.

Catalytic activityⁱ

Choloyl-CoA + glycine = CoA + glycocholate.2 Publications

Palmitoyl-CoA + H₂O = CoA + palmitate.1 Publication

Kineticsⁱ

Sites

Feature key	Position(s)	DescriptionActions	Length
Active siteⁱ	235	Charge relay systemCurated	1
Active siteⁱ	328	Charge relay systemCurated	1
Active siteⁱ	362	Charge relay systemCurated	1

GO - Molecular functionⁱ

carboxylic ester hydrolase activity Source: UniProtKB-KW
glycine N-choloyltransferase activity
Source: UniProtKB
Inferred from direct assayⁱ
- 12810727
- 8034703
long-chain acyl-CoA hydrolase activity
Source: UniProtKB
Inferred from direct assayⁱ
- 12810727
medium-chain acyl-CoA hydrolase activity
Source: UniProtKB
Inferred from direct assayⁱ
- 12810727
myristoyl-CoA hydrolase activity Source: UniProtKB-EC
N-acyltransferase activity
Source: MGI
Inferred from direct assayⁱ
- 12239217
palmitoyl-CoA hydrolase activity Source: UniProtKB-EC
signaling receptor binding
Source: UniProtKB
Inferred from physical interactionⁱ
- 20178365
transferase activity, transferring acyl groups Source: Reactome
very long chain acyl-CoA hydrolase activity
Source: UniProtKB
Inferred from direct assayⁱ
- 12810727

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

acyl-CoA metabolic process
Source: UniProtKB
Inferred from direct assayⁱ
- 12810727
animal organ regeneration Source: Ensembl
bile acid biosynthetic process
Source: UniProtKB
Inferred from direct assayⁱ
- 12810727
- 8034703
bile acid conjugation
Source: UniProtKB
Inferred from direct assayⁱ
- 12810727
- 8034703
bile acid metabolic process Source: Reactome
fatty acid metabolic process Source: GO_Central
glycine metabolic process
Source: UniProtKB
Inferred from direct assayⁱ
- 8034703
liver development Source: Ensembl
protein targeting to peroxisome Source: Reactome
taurine metabolic process
Source: UniProtKB
Inferred from direct assayⁱ
- 8034703

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Acyltransferase, Hydrolase, Serine esterase, Transferase
Biological process	Fatty acid metabolism, Lipid metabolism

Enzyme and pathway databases

BRENDAⁱ	2.3.1.65 2681
Reactomeⁱ	R-HSA-159418 Recycling of bile acids and salts R-HSA-193368 Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol R-HSA-9033241 Peroxisomal protein import
SABIO-RKⁱ	Q14032

Protein family/group databases

ESTHERⁱ	human-BAAT Acyl-CoA_Thioesterase
MEROPS protease database More...MEROPSⁱ	S09.A50

Chemistry databases

SwissLipidsⁱ	SLP:000001309

SwissLipidsⁱ

SLP:000001309

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Bile acid-CoA:amino acid N-acyltransferase1 Publication (EC:2.3.1.652 Publications) Short name: BACAT1 Publication Short name: BAT1 Publication Alternative name(s): Glycine N-choloyltransferase Long-chain fatty-acyl-CoA hydrolase1 Publication (EC:3.1.2.21 Publication)
Gene namesⁱ	Name:BAAT
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 9

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000136881.11
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:932 BAAT
MIMⁱ	602938 gene
neXtProtⁱ	NX_Q14032

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Cytoplasm

Pathology & Biotechⁱ

Involvement in diseaseⁱ

Familial hypercholanemia (FHCA)1 Publication

The disease may be caused by mutations affecting distinct genetic loci, including the gene represented in this entry.

Disease descriptionA disorder characterized by elevated serum bile acid concentrations, itching, and fat malabsorption.

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Natural variantⁱVAR_023737	76	M → V in FHCA. 1 PublicationCorresponds to variant dbSNP:rs28937579EnsemblClinVar.		1

Mutagenesis

Feature key	Position(s)	DescriptionActions	Length
Mutagenesisⁱ	235	C → A: Abolishes activity. 2 Publications	1
Mutagenesisⁱ	235	C → S: Lowers N-acyltransferase activity; enhanced thioesterase activity presumably dependent on the formation of a bile acid-enzyme covalent intermediate via a thioester bond. 2 Publications	1
Mutagenesisⁱ	328	D → A: Abolishes activity. 2 Publications	1
Mutagenesisⁱ	362	H → A: Abolishes activity. 2 Publications	1
Mutagenesisⁱ	372	C → A: Retains activity.	1
Mutagenesisⁱ	417	Q → K: Translocation to peroxisomes. 1 Publication	1

Keywords - Diseaseⁱ

Disease mutation

Organism-specific databases

DisGeNET More...DisGeNETⁱ	570
MalaCards human disease database More...MalaCardsⁱ	BAAT
MIMⁱ	607748 phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000136881
Orphanetⁱ	238475 Familial hypercholanemia
PharmGKBⁱ	PA25231

Chemistry databases

Drug and drug target database More...DrugBankⁱ	DB00145 Glycine DB05990 Obeticholic acid

DrugBankⁱ

DB00145 Glycine
DB05990 Obeticholic acid

Polymorphism and mutation databases

BioMutaⁱ	BAAT
DMDMⁱ	74739811

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
ChainⁱPRO_0000202159	1 – 418	Bile acid-CoA:amino acid N-acyltransferaseAdd BLAST		418

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Modified residueⁱ	125	PhosphoserineBy similarity		1
Modified residueⁱ	416	PhosphoserineBy similarity		1

Keywords - PTMⁱ

Phosphoprotein

Proteomic databases

MaxQB - The MaxQuant DataBase More...MaxQBⁱ	Q14032
PaxDbⁱ	Q14032
PeptideAtlas More...PeptideAtlasⁱ	Q14032
PRIDEⁱ	Q14032
ProteomicsDBⁱ	59802

PTM databases

CarbonylDBⁱ	Q14032
iPTMnetⁱ	Q14032
PhosphoSitePlusⁱ	Q14032

Expressionⁱ

Tissue specificityⁱ

Expressed in liver, gallbladder mucosa and pancreas.2 Publications

Gene expression databases

Bgeeⁱ	ENSG00000136881 Expressed in 54 organ(s), highest expression level in right lobe of liver
CleanExⁱ	HS_BAAT
Genevisibleⁱ	Q14032 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA021251 HPA021330

HPAⁱ

HPA021251
HPA021330

Interactionⁱ

Subunit structureⁱ

Monomer.1 Publication

Binary interactionsⁱ

With	Entry	#Exp.	IntAct	Notes
GOLGA8DP	Q0D2H9	5	EBI-8994378,EBI-10181276
GOLGA8G	Q08AF8	3	EBI-8994378,EBI-10181260

GO - Molecular functionⁱ

signaling receptor binding
Source: UniProtKB
Inferred from physical interactionⁱ
- 20178365

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

BioGridⁱ	107046, 5 interactors
IntActⁱ	Q14032, 21 interactors
Molecular INTeraction database More...MINTⁱ	Q14032
STRINGⁱ	9606.ENSP00000259407

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	Q14032
SMRⁱ	Q14032
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Sequence similaritiesⁱ

Belongs to the C/M/P thioester hydrolase family.Curated

Phylogenomic databases

eggNOGⁱ	ENOG410II3X Eukaryota COG1073 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000001046
HOGENOMⁱ	HOG000116219
HOVERGENⁱ	HBG000331
InParanoidⁱ	Q14032
KEGG Orthology (KO) More...KOⁱ	K00659
OMAⁱ	IPQVYRG
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G08KU
PhylomeDBⁱ	Q14032
TreeFamⁱ	TF314911

Family and domain databases

Gene3Dⁱ	3.40.50.1820, 1 hit
InterProⁱ	View protein in InterPro IPR029058 AB_hydrolase IPR016662 Acyl-CoA_thioEstase_long-chain IPR014940 BAAT_C IPR006862 Thio_Ohase/aa_AcTrfase
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF08840 BAAT_C, 1 hit PF04775 Bile_Hydr_Trans, 1 hit
PIRSFⁱ	PIRSF016521 Acyl-CoA_hydro, 1 hit
SUPFAMⁱ	SSF53474 SSF53474, 3 hits

Sequenceⁱ

Sequence statusⁱ: Complete.

Q14032-1 [UniParc]FASTA Add to basket

« Hide

        10         20         30         40         50
MIQLTATPVS ALVDEPVHIR ATGLIPFQMV SFQASLEDEN GDMFYSQAHY 
        60         70         80         90        100
RANEFGEVDL NHASSLGGDY MGVHPMGLFW SLKPEKLLTR LLKRDVMNRP 
       110        120        130        140        150
FQVQVKLYDL ELIVNNKVAS APKASLTLER WYVAPGVTRI KVREGRLRGA 
       160        170        180        190        200
LFLPPGEGLF PGVIDLFGGL GGLLEFRASL LASRGFASLA LAYHNYEDLP 
       210        220        230        240        250
RKPEVTDLEY FEEAANFLLR HPKVFGSGVG VVSVCQGVQI GLSMAIYLKQ 
       260        270        280        290        300
VTATVLINGT NFPFGIPQVY HGQIHQPLPH SAQLISTNAL GLLELYRTFE 
       310        320        330        340        350
TTQVGASQYL FPIEEAQGQF LFIVGEGDKT INSKAHAEQA IGQLKRHGKN 
       360        370        380        390        400
NWTLLSYPGA GHLIEPPYSP LCCASTTHDL RLHWGGEVIP HAAAQEHAWK 
       410 
EIQRFLRKHL IPDVTSQL

Length:418

Mass (Da):46,299

Last modified:November 1, 1996 - v1

Checksum:ⁱ4B290BAEE97F23B3

GO

Natural variant

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Natural variantⁱVAR_052303	20	R → Q3 PublicationsCorresponds to variant dbSNP:rs1572983EnsemblClinVar.		1
Natural variantⁱVAR_023737	76	M → V in FHCA. 1 PublicationCorresponds to variant dbSNP:rs28937579EnsemblClinVar.		1

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	L34081 mRNA Translation: AAC37550.1 CR541918 mRNA Translation: CAG46716.1 AL359893 Genomic DNA No translation available. CH471105 Genomic DNA Translation: EAW58943.1 BC009567 mRNA Translation: AAH09567.1 BC107424 mRNA Translation: AAI07425.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS6752.1
PIRⁱ	A53965
NCBI Reference Sequences More...RefSeqⁱ	NP_001121082.1, NM_001127610.1 NP_001692.1, NM_001701.3
UniGeneⁱ	Hs.284712

Genome annotation databases

Ensemblⁱ	ENST00000259407; ENSP00000259407; ENSG00000136881 ENST00000395051; ENSP00000378491; ENSG00000136881 ENST00000621712; ENSP00000484063; ENSG00000276559
GeneIDⁱ	570
KEGGⁱ	hsa:570
UCSC genome browser More...UCSCⁱ	uc010mtd.3 human

Keywords - Coding sequence diversityⁱ

Polymorphism

Protein	Similar proteins		Species	Score	Length	Source
Q14032	Bile acid Coenzyme A: amino acid N-acyltransferase (Glycine N-choloyltransferase), isoform CRA_b		HUMAN		175	UniRef90_Q14032
Bile acid-CoA:amino acid N-acyltransferase		PAPAN		418
Bile acid-CoA:amino acid N-acyltransferase		CHLSB		418
Bile acid-CoA:amino acid N-acyltransferase		MANLE		418
BAAT isoform 1		PONAB		418
+16

Protein	Similar proteins		Species	Score	Length	Source
Q14032	Bile acid-CoA:amino acid N-acyltransferase		MOUSE		420	UniRef50_Q14032
Acyl-coenzyme A amino acid N-acyltransferase 2		RAT		418
Bile acid Coenzyme A: amino acid N-acyltransferase (Glycine N-choloyltransferase), isoform CRA_b		HUMAN		175
Bile acid-CoA:amino acid N-acyltransferase		PAPAN		418
Bile acid-CoA:amino acid N-acyltransferase		CHLSB		418
+188

Entry informationⁱ

Entry nameⁱ	BAAT_HUMAN
Accessionⁱ	Q14032Primary (citable) accession number: Q14032 Secondary accession number(s): Q3B7W9, Q96L31
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	November 8, 2005
	Last sequence update:	November 1, 1996
	Last modified:	September 12, 2018
	This is version 146 of the entry and version 1 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousⁱ

Keywords - Technical termⁱ

Complete proteome, Direct protein sequencing, Reference proteome

UniProtKB

UniParc

Help

UniRef

Proteomes

Annotation systems

Supporting data

UniProtKB - Q14032 (BAAT_HUMAN)

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Bile acid-CoA:amino acid N-acyltransferase

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Miscellaneous

<p>This subsection of the ‘Function’ section describes biophysical and chemical properties, such as maximal absorption, kinetic parameters, pH dependence, redox potentials and temperature dependence.<p><a href='/help/biophysicochemical_properties' target='_top'>More...</a></p>Kineticsi

Sites

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

Protein family/group databases

Chemistry databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Other locations

Cytosol

Peroxisome

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Mutagenesis

Keywords - Diseasei

Organism-specific databases

Chemistry databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Keywords - PTMi

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

GO - Molecular functioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Phylogenomic databases

Family and domain databases

Natural variant

Sequence databases

Genome annotation databases

Keywords - Coding sequence diversityi

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

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Functionⁱ

Kineticsⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

Keywords - Diseaseⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

GO - Molecular functionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Coding sequence diversityⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ