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0THIM_HUMAN*   SwissProt (?) | Description Local Annotation Link Reference
General Information
Description3-ketoacyl-coa thiolase, mitochondrial (ec (beta- ketothiolase) (acetyl-coa acyltransferase) (mitochondrial 3-oxoacyl- coa thiolase) (t1).
SpeciesHomo sapiens (NCBI taxonomy ID: 9606)
Domain Architecture (Details)
InterPro domains unassigned to SynO:
Two different types of thiolase are found both in eukaryotes and in prokaryotes: acetoacetyl-CoA thiolase () and 3-ketoacyl-CoA thiolase (). 3-ketoacyl-CoA thiolase (also called thiolase I) has a broad chain-length specificity for its substrates and is involved in degradative pathways such as fatty acid beta-oxidation. Acetoacetyl-CoA thiolase (also called thiolase II) is specific for the thiolysis of acetoacetyl-CoA and involved in biosynthetic pathways such as poly beta-hydroxybutyrate synthesis or steroid biogenesis.In are two forms of 3-ketoacyl-CoA thiolase: one located in the mitochondrion and the other in peroxisomes.There are two conserved cysteine residues important for thiolase activity. The first located in the N-terminal section of the enzymes is involved in the formation of an acyl-enzyme intermediate; the second located at the C-terminal extremity is the active site base involved in deprotonation in the condensation reaction.Mammalian nonspecific lipid-transfer protein (nsL-TP) (also known as sterol carrier protein 2) is a protein which seems to exist in two different forms: a 14 Kd protein (SCP-2) and a larger 58 Kd protein (SCP-x). The former is found in the cytoplasm or the mitochondria and is involved in lipid transport; the latter is found in peroxisomes. The C-terminal part of SCP-x is identical to SCP-2 while the N-terminal portion is evolutionary related to thiolases .
SequencesProtein: THIM_HUMAN (397 aa)
mRNA: NM_006111
Local Annotation
Synapse Ontology
mitochondria are frequently observed in the vicinity of the synaptic vesicle clusters, in agreement with the ATP requirement of several steps of the vesicle cycle.
sdb:0118 mitochondria  (Evidence:keywords)
KO assignmentK07508
  Level 3 annotation:
    acetyl-CoA acyltransferase 2
  Level 2 annotation:
    Fatty acid elongation in mitochondria
    Fatty acid metabolism
    Bile acid biosynthesis
     leucine and isoleucine degradation
    Benzoate degradation via hydroxylation
Loci Structure (Details)Loci index, Chromosomal location, Length, Possible relational loci clusterExon1: 143 residues, 45563872-45564299Exon2: 53 residues, 45565564-45565719Exon3: 25 residues, 45567658-45567729Exon4: 45 residues, 45571837-45571967Exon5: 60 residues, 45572519-45572695Exon6: 51 residues, 45574647-45574795Exon7: 41 residues, 45576132-45576249Exon8: 45 residues, 45577833-45577962Exon9: 57 residues, 45583054-45583221Exon10: 23 residues, 45593836-45593900Exon11: 2 residues, -Jump to THIM_HUMAN  
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Loci Cluster (Details)Loci: 4341 45603183-45975176 ~-372K 17298(MYO5B)(-)Loci: 4340 45563872-45593900 ~-30K 17296(ACAA2)(-)Link out to UCSC