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Protein Page:
ACSF3 (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
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: Ligase; EC 6.2.1.-; EC 6.-.-.-
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 5 Y297-p FMAVPTIyTKLMEyy
0 4 Y303-p IyTKLMEyyDRHFTQ
0 2 Y304-p yTKLMEyyDRHFTQP
0 9 Y355-p GHTLLERyGMTEIGM
0 1 K439-a VFREYWNkPEETKSA
  mouse

 
Y297 FMAVPTVYSKLLDYY
Y303 VYSKLLDYYDKHFTQ
Y304 YSKLLDYYDKHFTQP
Y355 GHTLLERYGMTEIGM
K437 VFREYWDKPEETKSA
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