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Guidelines

MIRAGE

bile acid-CoA:amino acid N-acyltransferase

Summary

Gene Symbol

BAAT

Aliases

BAT
glycine N-choloyltransferase

Organism

Homo sapiens (human)

External Links

NCBI Gene

570

HGNC

932

KEGG Gene ID

hsa:570

PubChem

570

Alliance of Genome Resources

HGNC:932

JoGo

BAAT

TogoVar

BAAT

Gene Ontology (GO)

Displaying entries **1 - 5** of 16 in total

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GO Term	Evidence Code	PMID
liver development	IEA
bile acid conjugation	IDA
bile acid conjugation	IEA
glycine metabolic process	IDA
lipid metabolic process	IEA

GO Hierarchy

biological process

cellular process [inference]

metabolic process [inference]

sulfur compound metabolic process [inference]

acyl-CoA metabolic process

alkanesulfonate metabolic process [inference]

taurine metabolic process

phosphorus metabolic process [inference]

organophosphate metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process

biosynthetic process [inference]

small molecule biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

primary metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process

amino acid metabolic process [inference]

proteinogenic amino acid metabolic process [inference]

glycine metabolic process

lipid metabolic process

fatty acid metabolic process

steroid metabolic process [inference]

bile acid metabolic process

small molecule metabolic process [inference]

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process

fatty acid metabolic process

bile acid metabolic process

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

proteinogenic amino acid metabolic process [inference]

glycine metabolic process

small molecule biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process

purine-containing compound metabolic process [inference]

acyl-CoA metabolic process

developmental process [inference]

anatomical structure development [inference]

regeneration [inference]

animal organ regeneration

animal gross anatomical part developmental process [inference]

animal organ development [inference]

animal organ regeneration

gland development [inference]

liver development

Displaying entries **1 - 5** of 8 in total

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GO Term	Evidence Code	PMID
peroxisome	ISS
peroxisome	IDA
peroxisome	IBA
peroxisome	IEA
peroxisomal matrix	TAS

GO Hierarchy

cellular component

cellular anatomical structure [inference]

cytosol

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

microbody lumen [inference]

peroxisomal matrix

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

microbody [inference]

peroxisome

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

microbody [inference]

peroxisome

Displaying entries **1 - 5** of 20 in total

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GO Term	Evidence Code	PMID
protein binding	IPI
obsolete N-acyltransferase activity	IDA
obsolete N-acyltransferase activity	IBA
obsolete N-acyltransferase activity	IEA
acyltransferase activity	TAS

GO Hierarchy

molecular function

catalytic activity [inference]

transferase activity [inference]

acyltransferase activity

acyltransferase activity, transferring groups other than amino-acyl groups [inference]

glycine N-choloyltransferase activity

hydrolase activity [inference]

hydrolase activity, acting on ester bonds [inference]

thiolester hydrolase activity

acyl-CoA hydrolase activity [inference]

choloyl-CoA hydrolase activity

fatty acyl-CoA hydrolase activity

carboxylic ester hydrolase activity

deacylase activity [inference]

acyl-CoA hydrolase activity [inference]

choloyl-CoA hydrolase activity

fatty acyl-CoA hydrolase activity

binding [inference]

protein binding

Gene Ontology information from NCBI Gene and UniProt.

KEGG BRITE Database

Orthology

K00659

Name

bile acid-CoA:amino acid N-acyltransferase [EC:2.3.1.65 3.1.2.2]

References

Rhea

H₂O + (3S)-hydroxy-3-methylglutaryl-CoA

H⁺ + 3-hydroxy-3-methylglutarate + CoA

Enzyme

PMID

13563516

H₂O + choloyl-CoA

H⁺ + cholate + CoA

Enzyme

3.1.2.27

PMID

H₂O + chenodeoxycholoyl-CoA

H⁺ + chenodeoxycholate + CoA

Enzyme

3.1.2.27

PMID

H₂O + hexadecanoyl-CoA

H⁺ + CoA + hexadecanoate

Enzyme

3.1.2.2

PMID

glycine + choloyl-CoA

H⁺ + glycocholate + CoA

Enzyme

2.3.1.65

PMID

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0014667	disease of metabolism	Alliance of Genome Resources

The Human Phenotype Ontology

Displaying **all 9** entries
HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000952	Jaundice
HP:0001399	Hepatic failure
HP:0002240	Hepatomegaly
HP:0002748	Rickets
HP:0002908	Conjugated hyperbilirubinemia
HP:0003593	Infantile onset
HP:0003621	Juvenile onset
HP:0011463	Childhood onset

Displaying 1 entry
Disease ID	Disease Name
OMIM:619232	bile acid conjugation defect 1

PubChem Disease

MedGen Diseases

Bile acid conjugation defect 1

OMIM Phenotypes

BILE ACID CONJUGATION DEFECT 1; BACD1

KEGG Diseases

Familial hypercholanemia

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

LIPID MAPS Gene / Proteome Database

LMPD ID: LMP000423
Gene Name: bile acid CoA:amino acid N-acyltransferase

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International Collaboration

GlyCosmos is a member of the GlySpace Alliance together with GlyGen and Glycomics@ExPASy.

Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01

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GlyCosmos Portal v4.5.0

Last updated: April 6, 2026