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Protein Page:
ACSF3 (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
ACSF3 Catalyzes the initial reaction in intramitochondrial fatty acid synthesis, by activating malonate and methylmalonate, but not acetate, into their respective CoA thioester. May have some preference toward very-long-chain substrates. Defects in ACSF3 are the cause of combined malonic and methylmalonic aciduria (CMAMMA). A metabolic disease characterized by malonic and methylmalonic aciduria, with urinary excretion of much larger amounts of methylmalonic acid than malonic acid, in the presence of normal malonyl-CoA decarboxylase activity. Clinical features include coma, ketoacidosis, hypoglycemia, failure to thrive, microcephaly, dystonia, axial hypotonia and/or developmental delay, and neurologic manifestations including seizures, psychiatric disease and/or cognitive decline. Belongs to the ATP-dependent AMP-binding enzyme family. Note: This description may include information from UniProtKB.
Protein type: EC 6.-.-.-; Ligase; EC 6.2.1.-
Cellular Component: mitochondrion
Molecular Function: acid-thiol ligase activity; ATP binding
Biological Process: fatty acid metabolic process; fatty acid biosynthetic process
Reference #:  Q4G176 (UniProtKB)
Alt. Names/Synonyms: ACSF3; acyl-CoA synthetase family member 3; Acyl-CoA synthetase family member 3, mitochondrial; FLJ39242
Gene Symbols: ACSF3
Molecular weight: 64,130 Da
Basal Isoelectric point: 8.64  Predict pI for various phosphorylation states
Select Structure to View Below

ACSF3

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

 
0 1 E181 IYTGAVEEPAEVPVP
0 1 T205-p MIIYTSGtTGRPKGV
0 5 Y297-p FMAVPTIyTKLMEyy
0 4 Y303-p IyTKLMEyyDRHFTQ
0 2 Y304-p yTKLMEyyDRHFTQP
0 1 R306 KLMEyyDRHFTQPHA
0 9 Y355-p GHTLLERyGMTEIGM
0 1 R396 IVSENPQREACSYTI
0 1 K421 VTPGFEEKEGELLVR
0 2 K439-ac VFREYWNkPEETKSA
0 1 Y466-p VVFKDGQyWIRGRTS
0 1 K534 SLSHRELKEWARNVL
0 1 K534-sc SLSHRELkEWARNVL
0 1 Y544-p ARNVLAPyAVPSELV
0 1 K567 QMGKIDKKALIRHFH
0 1 R571 IDKKALIRHFHPS__
  mouse

 
K181-ac VYHGATEkPTEQPVE
T205 MIFYTSGTTGRPKGA
Y297 FMAVPTVYSKLLDYY
Y303 VYSKLLDYYDkHFTQ
Y304 YSKLLDYYDkHFTQP
K306-ac KLLDYYDkHFTQPHV
Y355 GHTLLERYGMTEIGM
K395-ac IISENPQkGSPYIIH
K419-ac VTPGFEEkEGELLVR
K437-ac VFREYWDkPEETKSA
Y464 AVFKDARYWIRGRTS
K532-ac SLSHGDLkEWARGVL
K532 SLSHGDLKEWARGVL
Y542 ARGVLAPYAVPSELL
K565-ac QMGKVNKkELLkQLY
K569-ac VNKkELLkQLYPSGQ
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