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Protein Page:
ACAT1 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

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: Carbohydrate Metabolism - pyruvate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Amino Acid Metabolism - tryptophan; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Lipid Metabolism - synthesis and degradation of ketone bodies; Carbohydrate Metabolism - propanoate; Carbohydrate Metabolism - butanoate; Acetyltransferase; Mitochondrial; Amino Acid Metabolism - lysine degradation; Lipid Metabolism - fatty acid; EC 2.3.1.9
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; ketone body metabolic process; brain development; cellular lipid 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 K36 VERSYVSKPTLKEVV
0 1 K66 LSLLPATKLGSIAIQ
0 1 K66 LSLLPATKLGSIAIQ
0 1 S69 LPATKLGSIAIQGAI
0 1 K78 AIQGAIEKAGIPkEE
0 2 K83-ac IEKAGIPkEEVKEAy
0 1 K83 IEKAGIPKEEVKEAy
0 1 K87 GIPkEEVKEAyMGNV
0 1 K87 GIPkEEVKEAyMGNV
0 2 Y90-p kEEVKEAyMGNVLQG
0 1 T120 LPISTPCTTINkVCA
0 1 T121 PISTPCTTINkVCAS
0 11 K124-ac TPCTTINkVCASGMk
0 1 S128 TINkVCASGMkAIMM
0 3 K131-ac kVCASGMkAIMMAsQ
0 5 S137-p MkAIMMAsQSLMCGH
0 1 S155 MVAGGMESMSNVPYV
0 1 S157 AGGMESMSNVPYVMN
0 1 Y161 ESMSNVPYVMNRGST
0 27 K174-ac STPYGGVkLEDLIVk
0 2 K174-ub STPYGGVkLEDLIVk
0 2 K174-sc STPYGGVkLEDLIVk
0 4 K181-ac kLEDLIVkDGLTDVY
0 3 K181-ub kLEDLIVkDGLTDVY
0 1 K181 kLEDLIVKDGLTDVY
0 11 K190-ac GLTDVYNkIHMGSCA
0 1 K190 GLTDVYNKIHMGSCA
0 110 K202-ac SCAENTAkkLNIARN
0 1 K202-sc SCAENTAkkLNIARN
0 1 K203-ac CAENTAkkLNIARNE
0 1 A207 TAkkLNIARNEQDAy
0 6 Y214-p ARNEQDAyAINSytR
0 13 Y219-p DAyAINSytRsKAAW
0 1 T220-p AyAINSytRsKAAWE
0 1 S222-p AINSytRsKAAWEAG
0 4 K223 INSytRsKAAWEAGk
0 2 K223 INSytRsKAAWEAGk
0 6 K230-ac KAAWEAGkFGNEVIP
0 2 K230 KAAWEAGKFGNEVIP
0 1 T241 EVIPVTVTVkGQPDV
0 1 K243 IPVTVTVKGQPDVVV
0 2 K243-sc IPVTVTVkGQPDVVV
0 4 Q245 VTVTVkGQPDVVVkE
0 1 Q245 VTVTVkGQPDVVVkE
0 2 Q245 VTVTVkGQPDVVVkE
0 4 K251-ac GQPDVVVkEDEEYkR
0 2 K251-sc GQPDVVVkEDEEYkR
0 68 K257-ac VkEDEEYkRVDFskV
0 1 K257 VkEDEEYKRVDFskV
0 1 S262-p EYkRVDFskVPKLKT
0 4 K263-ac YkRVDFskVPKLKTV
0 1 K263 YkRVDFsKVPKLKTV
0 2 K263-sc YkRVDFskVPKLKTV
0 1 K266 VDFskVPKLKTVFQk
0 1 K268 FskVPKLKTVFQkEN
0 2 K273 KLKTVFQKENGTVtA
0 2 K273-sc KLKTVFQkENGTVtA
0 1 T279-p QkENGTVtAANAStL
0 1 T285-p VtAANAStLNDGAAA
0 44 K302-ac LMTADAAkRLNVTPL
0 1 K302-sc LMTADAAkRLNVTPL
0 17 T307 AAkRLNVTPLARIVA
0 1 T307 AAkRLNVTPLARIVA
0 2 T307 AAkRLNVTPLARIVA
0 4 Y331 DFPIAPVYAASMVLK
0 2 M335 APVYAASMVLKDVGL
0 3 K338 YAASMVLKDVGLKKE
0 1 K338 YAASMVLKDVGLKKE
0 2 K338 YAASMVLKDVGLKKE
0 2 K343 VLKDVGLKKEDIAMW
0 1 K373 MLEIDPQKVNINGGA
0 1 K373 MLEIDPQKVNINGGA
1 1 Y407-p HALKQGEyGLASICN
  ACAT1 iso2  
K36 VERSYVSKPTLKEVV
K66 LSLLPATKLGSIAIQ
K66 LSLLPATKLGSIAIQ
S69 LPATKLGSIAIQGAI
K78 AIQGAIEKAGIPKEE
K83 IEKAGIPKEEVKEAY
K83 IEKAGIPKEEVKEAY
K87 GIPKEEVKEAYMGNV
K87 GIPKEEVKEAYMGNV
Y90 KEEVKEAYMGNVLQG
T120 LPISTPCTTINKVCA
T121 PISTPCTTINKVCAs
K124 TPCTTINKVCAsGMK
S128-p TINKVCAsGMKAIMM
K131 KVCAsGMKAIMMASQ
S137 MKAIMMASQSLMCGH
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  mouse

 
K33-sc LERSYASkPTLNEVV
K63-ac LASQPATkLGtAAIQ
K63-sc LASQPATkLGtAAIQ
T66-p QPATkLGtAAIQGAI
K75-ac AIQGAIEkAGIPkEE
K80-ac IEkAGIPkEEVkEVy
K80-sc IEkAGIPkEEVkEVy
K84-ac GIPkEEVkEVyMGNV
K84-ub GIPkEEVkEVyMGNV
Y87-p kEEVkEVyMGNVIQG
T117-p LPISTPCttVNKVCA
T118-p PISTPCttVNKVCAS
K121 TPCttVNKVCASGMK
S125 tVNKVCASGMKAIMM
K128 KVCASGMKAIMMASQ
S134 MKAIMMASQSLMCGH
S152-p MVAGGMEsMsNVPyV
S154-p AGGMEsMsNVPyVMS
Y158-p EsMsNVPyVMSRGAT
K171-ac ATPYGGVkLEDLIVk
K171-ub ATPYGGVkLEDLIVk
K171-sc ATPYGGVkLEDLIVk
K178-ac kLEDLIVkDGLTDVY
K178-ub kLEDLIVkDGLTDVY
K178-sc kLEDLIVkDGLTDVY
K187-ac GLTDVYNkIHMGNCA
K187-sc GLTDVYNkIHMGNCA
K199-ac NCAENTAkKMNIsRQ
K199 NCAENTAKKMNIsRQ
K200 CAENTAkKMNIsRQE
S204-p TAkKMNIsRQEQDTY
Y211 sRQEQDTYALSSYTR
Y216 DTYALSSYTRSkEAW
T217 TYALSSYTRSkEAWD
S219 ALSSYTRSkEAWDAG
K220-ac LSSYTRSkEAWDAGk
K220-sc LSSYTRSkEAWDAGk
K227-ac kEAWDAGkFASEITP
K227-sc kEAWDAGkFASEITP
S238-p EITPITIsVkGkPDV
K240-ac TPITIsVkGkPDVVV
K240-sc TPITIsVkGkPDVVV
K242-ac ITIsVkGkPDVVVkE
K242-ub ITIsVkGkPDVVVkE
K242-sc ITIsVkGkPDVVVkE
K248-ac GkPDVVVkEDEEYkR
K248-sc GkPDVVVkEDEEYkR
K254-ac VkEDEEYkRVDFSkV
K254-sc VkEDEEYkRVDFSkV
S259 EYkRVDFSkVPkLkT
K260-ac YkRVDFSkVPkLkTV
K260-ub YkRVDFSkVPkLkTV
K260-sc YkRVDFSkVPkLkTV
K263-ac VDFSkVPkLkTVFQk
K265-ac FSkVPkLkTVFQkEN
K270-ac kLkTVFQkENGTITA
K270-sc kLkTVFQkENGTITA
T276 QkENGTITAANASTL
T282 ITAANASTLNDGAAA
Q299 LMTAEAAQRLNVkPL
Q299 LMTAEAAQRLNVkPL
K304-ac AAQRLNVkPLARIAA
K304-ub AAQRLNVkPLARIAA
K304-sc AAQRLNVkPLARIAA
Y328-p DFPLAPAyAVPkVLk
K332-ac APAyAVPkVLkYAGL
K335-ac yAVPkVLkYAGLkKE
K335-ub yAVPkVLkYAGLkKE
K335-sc yAVPkVLkYAGLkKE
K340-ac VLkYAGLkKEDIAMW
K370-ac MLEIDPQkVNIHGGA
K370-ub MLEIDPQkVNIHGGA
F404 HALKPGEFGLASICN
  rat

 
K33 LGRSYASKPTLNDVV
K63 LASQPATKLGTIAIQ
K63 LASQPATKLGTIAIQ
T66 QPATKLGTIAIQGAI
K75 AIQGAIEKAGIPKEE
K80 IEKAGIPKEEVKEVY
K80 IEKAGIPKEEVKEVY
K84 GIPKEEVKEVYMGNV
K84 GIPKEEVKEVYMGNV
Y87 KEEVKEVYMGNVIQG
T117 LPIATPCTTVNKVCA
T118 PIATPCTTVNKVCAS
K121 TPCTTVNKVCASGMK
S125 TVNKVCASGMKAIMM
K128 KVCASGMKAIMMASQ
S134 MKAIMMASQSLMCGH
S152 MVAGGMESMSNVPYV
S154 AGGMESMSNVPYVMS
Y158 ESMSNVPYVMSRGAT
K171 ATPYGGVKLEDLIVK
K171 ATPYGGVKLEDLIVK
K171 ATPYGGVKLEDLIVK
K178 KLEDLIVKDGLTDVY
K178 KLEDLIVKDGLTDVY
K178 KLEDLIVKDGLTDVY
K187 GLTDVYNKIHMGNCA
K187 GLTDVYNKIHMGNCA
K199 NCAENTAKKLSISRE
K199 NCAENTAKKLSISRE
K200 CAENTAKKLSISREE
S204 TAKKLSISREEQDKY
Y211 SREEQDKYAIGSYTR
Y216 DKYAIGSYTRSKEAW
T217 KYAIGSYTRSKEAWD
S219 AIGSYTRSKEAWDAG
K220 IGSYTRSKEAWDAGk
K220 IGSYTRSKEAWDAGk
K227-ac KEAWDAGkFANEITP
K227 KEAWDAGKFANEITP
S238 EITPITISVKGKPDV
K240 TPITISVKGKPDVVV
K240 TPITISVKGKPDVVV
K242 ITISVKGKPDVVVKE
K242 ITISVKGKPDVVVKE
K242 ITISVKGKPDVVVKE
K248 GKPDVVVKEDEEYKR
K248 GKPDVVVKEDEEYKR
K254 VKEDEEYKRVDFSKV
K254 VKEDEEYKRVDFSKV
S259 EYKRVDFSKVPKLKT
K260 YKRVDFSKVPKLKTV
K260 YKRVDFSKVPKLKTV
K260 YKRVDFSKVPKLKTV
K263 VDFSKVPKLKTVFQK
K265 FSKVPKLKTVFQKEN
K270 KLKTVFQKENGTVTA
K270 KLKTVFQKENGTVTA
T276 QKENGTVTAANASTL
T282 VTAANASTLNDGAAA
Q299 LMTAEAAQRLKVKPL
Q299 LMTAEAAQRLKVKPL
K304 AAQRLKVKPLARIAA
K304 AAQRLKVKPLARIAA
K304 AAQRLKVKPLARIAA
Y328 DFPLAPAYAVPKVLK
K332 APAYAVPKVLKYAGL
K335 YAVPKVLKYAGLKKE
K335 YAVPKVLKYAGLKKE
K335 YAVPKVLKYAGLKKE
K340 VLKYAGLKKEDIAMW
K370 MLEIDPQKVNVHGGA
K370 MLEIDPQKVNVHGGA
F404 HALKQGEFGLASICN
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