Gene/Proteome Database (LMPD)

LMPD ID
LMP000423
Gene ID
570
Species
Homo sapiens (Human)
Gene Name
bile acid CoA:amino acid N-acyltransferase
Gene Symbol
BAAT
Synonyms
BACAT; BAT
Alternate Names
bile acid-CoA:amino acid N-acyltransferase; long-chain fatty-acyl-CoA hydrolase; bile acid CoA: amino acid N-acyltransferase (glycine N-choloyltransferase); bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase)
Chromosome
9
Map Location
9q22.3
EC Number
2.3.1.65
Summary
The protein encoded by this gene is a liver enzyme that catalyzes the transfer of C24 bile acids from the acyl-CoA thioester to either glycine or taurine, the second step in the formation of bile acid-amino acid conjugates. The bile acid conjugates then act as a detergent in the gastrointestinal tract, which enhances lipid and fat-soluble vitamin absorption. Defects in this gene are a cause of familial hypercholanemia (FHCA). Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]
Orthologs
View orthologs and multiple alignments for BAAT

Proteins

bile acid-CoA:amino acid N-acyltransferase
Refseq ID NP_001121082
Protein GI 189083866
UniProt ID Q14032
mRNA ID NM_001127610
Length 418
RefSeq Status REVIEWED
MIQLTATPVSALVDEPVHIRATGLIPFQMVSFQASLEDENGDMFYSQAHYRANEFGEVDLNHASSLGGDYMGVHPMGLFWSLKPEKLLTRLLKRDVMNRPFQVQVKLYDLELIVNNKVASAPKASLTLERWYVAPGVTRIKVREGRLRGALFLPPGEGLFPGVIDLFGGLGGLLEFRASLLASRGFASLALAYHNYEDLPRKPEVTDLEYFEEAANFLLRHPKVFGSGVGVVSVCQGVQIGLSMAIYLKQVTATVLINGTNFPFGIPQVYHGQIHQPLPHSAQLISTNALGLLELYRTFETTQVGASQYLFPIEEAQGQFLFIVGEGDKTINSKAHAEQAIGQLKRHGKNNWTLLSYPGAGHLIEPPYSPLCCASTTHDLRLHWGGEVIPHAAAQEHAWKEIQRFLRKHLIPDVTSQL
bile acid-CoA:amino acid N-acyltransferase
Refseq ID NP_001692
Protein GI 4502351
UniProt ID Q14032
mRNA ID NM_001701
Length 418
RefSeq Status REVIEWED
Protein sequence is identical to GI:189083866 (mRNA isoform)

Gene Information

Entrez Gene ID
570
Gene Name
bile acid CoA:amino acid N-acyltransferase
Gene Symbol
BAAT
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005829 IDA:UniProtKB C cytosol
GO:0005782 TAS:Reactome C peroxisomal matrix
GO:0005777 IDA:UniProtKB C peroxisome
GO:0016410 IDA:MGI F N-acyltransferase activity
GO:0052689 IEA:UniProtKB-KW F carboxylic ester hydrolase activity
GO:0047963 IDA:UniProtKB F glycine N-choloyltransferase activity
GO:0052816 IDA:UniProtKB F long-chain acyl-CoA hydrolase activity
GO:0052815 IDA:UniProtKB F medium-chain acyl-CoA hydrolase activity
GO:0016290 IEA:UniProtKB-EC F palmitoyl-CoA hydrolase activity
GO:0005102 IPI:UniProtKB F receptor binding
GO:0052817 IDA:UniProtKB F very long chain acyl-CoA hydrolase activity
GO:0006637 IDA:UniProtKB P acyl-CoA metabolic process
GO:0015721 TAS:Reactome P bile acid and bile salt transport
GO:0006699 IDA:UniProtKB P bile acid biosynthetic process
GO:0002152 IDA:UniProtKB P bile acid conjugation
GO:0008206 TAS:Reactome P bile acid metabolic process
GO:0006631 IEA:UniProtKB-KW P fatty acid metabolic process
GO:0006544 IDA:UniProtKB P glycine metabolic process
GO:0001889 IEA:Ensembl P liver development
GO:0031100 IEA:Ensembl P organ regeneration
GO:0044281 TAS:Reactome P small molecule metabolic process
GO:0019530 IDA:UniProtKB P taurine metabolic process

KEGG Pathway Links

KEGG Pathway ID Description
hsa04976 Bile secretion
hsa04146 Peroxisome
hsa00120 Primary bile acid biosynthesis

Domain Information

InterPro Annotations

Accession Description
IPR006862 Acyl-CoA thioester hydrolase/bile acid-CoA amino acid N-acetyltransferase
IPR016662 Acyl-CoA thioesterase, long chain
IPR029058 Alpha/Beta hydrolase fold
IPR014940 BAAT/Acyl-CoA thioester hydrolase C-terminal

UniProt Annotations

Entry Information

Gene Name
bile acid CoA:amino acid N-acyltransferase
Protein Entry
BAAT_HUMAN
UniProt ID
Q14032
Species
Human

Comments

Comment Type Description
Biophysicochemical Properties Kinetic parameters: KM=1.1 mM for taurine toward choloyl-CoA ; KM=2.2 mM for 2-fluoro-beta-alanine toward choloyl-CoA ; KM=5.8 mM for glycine toward choloyl-CoA ; KM=19.3 uM for arachidoyl-CoA ; Vmax=0.33 umol/min/mg enzyme with taurine as substrate for acyltransferase activity ; Vmax=0.19 umol/min/mg enzyme with 2-fluoro-beta-alanine as substrate for acyltransferase activity ; Vmax=0.77 umol/min/mg enzyme with glycine as substrate for acyltransferase activity ; Vmax=223 nmol/min/mg enzyme with arachidoyl-CoA as substrate for acyl-CoA thioesterase activity ;
Catalytic Activity Choloyl-CoA + glycine = CoA + glycocholate.
Catalytic Activity Palmitoyl-CoA + H(2)O = CoA + palmitate.
Disease Familial hypercholanemia (FHCA) [MIM
Function 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.
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.
Similarity Belongs to the C/M/P thioester hydrolase family.
Subcellular Location Cytoplasm .
Subunit Monomer.
Tissue Specificity Expressed in liver, gallbladder mucosa and pancreas. {ECO

Identical and Related Proteins

Unique RefSeq proteins for LMP000423 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
189083866 RefSeq NP_001121082 418 bile acid-CoA:amino acid N-acyltransferase

Identical Sequences to LMP000423 proteins

Reference Database Accession Length Protein Name
GI:189083866 EMBL CAG46716.1 418 BAAT, partial [Homo sapiens]
GI:189083866 GenBank AAC37550.1 418 bile acid CoA: Amino acid N-acyltransferase [Homo sapiens]
GI:189083866 GenBank ACM80615.1 418 Sequence 6113 from patent US 6812339
GI:189083866 GenBank AHE01119.1 418 Sequence 56035 from patent US 8586006
GI:189083866 RefSeq NP_001692.1 418 bile acid-CoA:amino acid N-acyltransferase [Homo sapiens]
GI:189083866 SwissProt Q14032.1 418 RecName: Full=Bile acid-CoA:amino acid N-acyltransferase; Short=BACAT; Short=BAT; AltName: Full=Glycine N-choloyltransferase; AltName: Full=Long-chain fatty-acyl-CoA hydrolase [Homo sapiens]

Related Sequences to LMP000423 proteins

Reference Database Accession Length Protein Name
GI:189083866 GenBank AAH09567.1 418 Bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase) [Homo sapiens]
GI:189083866 GenBank AAX36764.1 419 bile acid coenzyme A: amino acid N-acyltransferase, partial [synthetic construct]
GI:189083866 GenBank AAI07425.1 418 Bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase) [Homo sapiens]
GI:189083866 GenBank EAW58943.1 418 bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase), isoform CRA_a [Homo sapiens]
GI:189083866 GenBank EAW58945.1 418 bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase), isoform CRA_a [Homo sapiens]
GI:189083866 GenBank ABM82836.1 418 bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase) [synthetic construct]