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Protein Page:
ACAT1 (human)
p Phosphorylation
a Acetylation
m Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
u Ubiquitination
s Sumoylation
n Neddylation
gl O-GlcNAc
ga O-GalNAc
h Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage

Overview
ACAT1 Plays a major role in ketone body metabolism. Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD); also known as alpha- methylacetoaceticaciduria. 3KTD is an inborn error of isoleucine catabolism characterized by intermittent ketoacidotic attacks associated with unconsciousness. Some patients die during an attack or are mentally retarded. Urinary excretion of 2-methyl-3- hydroxybutyric acid, 2-methylacetoacetic acid, triglylglycine, butanone is increased. It seems likely that the severity of this disease correlates better with the environmental or acquired factors than with the ACAT1 genotype. Belongs to the thiolase family. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - lysine degradation; Amino Acid Metabolism - tryptophan; Lipid Metabolism - fatty acid; Mitochondrial; Carbohydrate Metabolism - pyruvate; EC 2.3.1.9; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Acetyltransferase; Carbohydrate Metabolism - propanoate; Lipid Metabolism - synthesis and degradation of ketone bodies
Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane
Molecular Function: protein homodimerization activity; enzyme binding; acetyl-CoA C-acetyltransferase activity; metal ion binding; coenzyme binding
Biological Process: response to starvation; ketone body catabolic process; response to hormone stimulus; ketone body biosynthetic process; branched chain family amino acid catabolic process; brain development; cellular lipid metabolic process; ketone body metabolic process; liver development; response to organic cyclic substance; protein homooligomerization
Reference #:  P24752 (UniProtKB)
Alt. Names/Synonyms: ACAT; ACAT1; acetoacetyl Coenzyme A thiolase; Acetoacetyl-CoA thiolase; Acetyl-CoA acetyltransferase, mitochondrial; acetyl-Coenzyme A acetyltransferase 1; MAT; mitochondrial acetoacetyl-CoA thiolase; T2; THIL
Gene Symbols: ACAT1
Molecular weight: 45,200 Da
Basal Isoelectric point: 8.98  Predict pI for various phosphorylation states
Select Structure to View Below

ACAT1

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

► Hide Isoforms
 
0 1 K78 AIQGAIEKAGIPkEE
0 2 K83-a IEKAGIPkEEVKEAy
0 1 K87 GIPkEEVKEAyMGNV
0 2 Y90-p kEEVKEAyMGNVLQG
0 1 T120 LPISTPCTTINkVCA
0 1 T121 PISTPCTTINkVCAS
0 11 K124-a TPCTTINkVCASGMk
0 1 S128 TINkVCASGMkAIMM
0 3 K131-a kVCASGMkAIMMAsQ
0 5 S137-p MkAIMMAsQSLMCGH
0 26 K174-a STPYGGVkLEDLIVk
0 2 K174-u STPYGGVkLEDLIVk
0 3 K181-a kLEDLIVkDGLTDVY
0 3 K181-u kLEDLIVkDGLTDVY
0 11 K190-a GLTDVYNkIHMGSCA
0 110 K202-a SCAENTAkKLNIARN
0 1 A207 TAkKLNIARNEQDAy
0 6 Y214-p ARNEQDAyAINSyTR
0 13 Y219-p DAyAINSyTRSKAAW
0 3 K223 INSyTRSKAAWEAGK
0 5 K230 KAAWEAGKFGNEVIP
0 1 T241 EVIPVTVTVKGQPDV
0 3 Q245 VTVTVKGQPDVVVkE
0 1 Q245 VTVTVKGQPDVVVkE
0 3 K251-a GQPDVVVkEDEEYkR
0 68 K257-a VkEDEEYkRVDFSkV
0 4 K263-a YkRVDFSkVPKLKTV
0 1 K263 YkRVDFSKVPKLKTV
0 2 K273 KLKTVFQKENGTVTA
0 43 K302-a LMTADAAkRLNVTPL
0 17 T307 AAkRLNVTPLARIVA
0 1 T307 AAkRLNVTPLARIVA
0 4 Y331 DFPIAPVYAASMVLK
0 1 M335 APVYAASMVLKDVGL
0 3 K338 YAASMVLKDVGLKKE
0 1 K338 YAASMVLKDVGLKKE
0 2 K343 VLKDVGLKKEDIAMW
0 1 K373 MLEIDPQKVNINGGA
1 1 Y407-p HALKQGEyGLASICN
  ACAT1 iso2  
K78 AIQGAIEKAGIPKEE
K83 IEKAGIPKEEVKEAY
K87 GIPKEEVKEAYMGNV
Y90 KEEVKEAYMGNVLQG
T120 LPISTPCTTINKVCA
T121 PISTPCTTINKVCAs
K124 TPCTTINKVCAsGMK
S128-p TINKVCAsGMKAIMM
K131 KVCAsGMKAIMMASQ
S137 MKAIMMASQSLMCGH
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  mouse

 
K75-a AIQGAIEkAGIPkEE
K80-a IEkAGIPkEEVkEVy
K84-u GIPkEEVkEVyMGNV
Y87-p kEEVkEVyMGNVIQG
T117-p LPISTPCttVNKVCA
T118-p PISTPCttVNKVCAS
K121 TPCttVNKVCASGMK
S125 tVNKVCASGMKAIMM
K128 KVCASGMKAIMMASQ
S134 MKAIMMASQSLMCGH
K171-a ATPYGGVkLEDLIVk
K171-u ATPYGGVkLEDLIVk
K178-a kLEDLIVkDGLTDVY
K178-u kLEDLIVkDGLTDVY
K187-a GLTDVYNkIHMGNCA
K199-a NCAENTAkKMNIsRQ
S204-p TAkKMNIsRQEQDTY
Y211 sRQEQDTYALSSYTR
Y216 DTYALSSYTRSkEAW
K220-a LSSYTRSkEAWDAGk
K227-a kEAWDAGkFASEITP
S238-p EITPITIsVKGkPDV
K242-a ITIsVKGkPDVVVkE
K242-u ITIsVKGkPDVVVkE
K248-a GkPDVVVkEDEEYkR
K254-a VkEDEEYkRVDFSkV
K260-a YkRVDFSkVPKLKTV
K260-u YkRVDFSkVPKLKTV
K270-a KLKTVFQkENGTITA
Q299 LMTAEAAQRLNVkPL
K304-a AAQRLNVkPLARIAA
K304-u AAQRLNVkPLARIAA
Y328-p DFPLAPAyAVPkVLk
K332-a APAyAVPkVLkYAGL
K335-a yAVPkVLkYAGLkKE
K335-u yAVPkVLkYAGLkKE
K340-a VLkYAGLkKEDIAMW
K370-u MLEIDPQkVNIHGGA
F404 HALKPGEFGLASICN
  rat

 
K75 AIQGAIEKAGIPKEE
K80 IEKAGIPKEEVKEVY
K84 GIPKEEVKEVYMGNV
Y87 KEEVKEVYMGNVIQG
T117 LPIATPCTTVNKVCA
T118 PIATPCTTVNKVCAS
K121 TPCTTVNKVCASGMK
S125 TVNKVCASGMKAIMM
K128 KVCASGMKAIMMASQ
S134 MKAIMMASQSLMCGH
K171 ATPYGGVKLEDLIVK
K171 ATPYGGVKLEDLIVK
K178 KLEDLIVKDGLTDVY
K178 KLEDLIVKDGLTDVY
K187 GLTDVYNKIHMGNCA
K199 NCAENTAKKLSISRE
S204 TAKKLSISREEQDKY
Y211 SREEQDKYAIGSYTR
Y216 DKYAIGSYTRSKEAW
K220 IGSYTRSKEAWDAGk
K227-a KEAWDAGkFANEITP
S238 EITPITISVKGKPDV
K242 ITISVKGKPDVVVKE
K242 ITISVKGKPDVVVKE
K248 GKPDVVVKEDEEYKR
K254 VKEDEEYKRVDFSKV
K260 YKRVDFSKVPKLKTV
K260 YKRVDFSKVPKLKTV
K270 KLKTVFQKENGTVTA
Q299 LMTAEAAQRLKVKPL
K304 AAQRLKVKPLARIAA
K304 AAQRLKVKPLARIAA
Y328 DFPLAPAYAVPKVLK
K332 APAYAVPKVLKYAGL
K335 YAVPKVLKYAGLKKE
K335 YAVPKVLKYAGLKKE
K340 VLKYAGLKKEDIAMW
K370 MLEIDPQKVNVHGGA
F404 HALKQGEFGLASICN
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