Disorders in ketone body synthesis (Homo sapiens)

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32Cytosol 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency3-Hydroxy-3- methylglutaryl-CoA synthase deficiencyMethylacetoacetyl- CoA thiolase deficiencyAcetoacetyl-CoACoenzym A3-hydroxybutyric acidTricarboxylicacid cycleHMGCLBDH1ACAT1Acetyl-CoAHMGCS2acetoacetateacetoneLeucineCoASHfatty acidsNAD+3-hydroxy-3-methylglutaryl-coenzyme AMitochondrial long chain fatty acid beta-oxidationH+NADHAcetyl-CoAH+Mitochondrial matrix Carbon DioxideLeucine, isoleucine and valine metabolismDiseaseCatalysisPathwayProteinMetaboliteConversionConnection to diseaseLegendProtein with specficlocalisation information


Description

Disorders in ketone body synthesis

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Quality Tags

Ontology Terms

 

Bibliography

  1. Morris, AMM; ''Inborn metabolic diseases''; ISBN: 978-3-642-15720-2, 2012
  2. Blau, Nenad, Duran, Marinus, Gibson, K. Michael, Dionisi-Vici, Carlos; ''Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases''; 978-3-642-40337-8, 2014 DOI Scholia

History

View all...
CompareRevisionActionTimeUserComment
128229view13:32, 29 January 2024EweitzStandardize case
128228view13:29, 29 January 2024EweitzStandardize case, trim node padding
128227view13:24, 29 January 2024EweitzSoften color, use standard font weight in disease nodes
128226view13:22, 29 January 2024EweitzUse standard pathway node shape
125326view22:17, 31 January 2023LarsgwAdd ISBN, DOI to references
122904view12:16, 31 May 2022DeSlUpdated description to include info from WP311
122903view12:12, 31 May 2022DeSlmerged content from WP311 into this PW
122517view12:48, 13 April 2022DeSlRemoved unneccessary element from legend
122516view12:47, 13 April 2022DeSlUpdate description
122515view12:45, 13 April 2022DeSlConnecrted unconnected line in legend to graphical line
122368view11:45, 24 March 2022SamDrabbefixed interaction
122360view10:43, 23 March 2022SamDrabbedata node change pyruvate metabolism
122359view10:31, 23 March 2022SamDrabbeadded glucosylation pathway
122358view10:26, 23 March 2022SamDrabbeadded mevalonate biosynthesis
122357view10:21, 23 March 2022SamDrabbeData Node HMGCL changed
122356view10:20, 23 March 2022SamDrabbedescription ACAT1 changed
122355view10:19, 23 March 2022SamDrabbeData Node ACAT1 changed
122354view10:01, 23 March 2022SamDrabbeModified description
122353view09:56, 23 March 2022SamDrabbeModified description
122246view12:27, 16 March 2022SamDrabbeadded sterol biosynthesis
122233view10:32, 16 March 2022SamDrabbeOntology Term : 'citric acid cycle pathway' added !
122229view10:26, 16 March 2022SamDrabbeOntology Term : 'fatty acid metabolic pathway' added !
122227view10:25, 16 March 2022SamDrabbepathways added
122223view10:02, 16 March 2022SamDrabbeDataNode change
122221view10:00, 16 March 2022SamDrabbechange BDH1 DataNode
122073view23:01, 9 March 2022DeSlremoved PMID:29630231 from GPML
122063view13:57, 9 March 2022SamDrabbeAcetoacetyl-CoA annotation updated
122061view13:56, 9 March 2022SamDrabbeAcetyl-CoA annotation updated
122051view12:56, 9 March 2022SamDrabbeconversion adjusted
122048view12:33, 9 March 2022SamDrabbeliterature
122003view09:20, 9 March 2022SamDrabbeliterature change
121807view15:30, 6 March 2022SamDrabbeLegend added
121800view14:09, 6 March 2022EgonwNot an conversion
121763view12:22, 3 March 2022DeSlMore small layout changes
121762view12:12, 3 March 2022DeSlMore layout and condensation of DataNodes
121761view12:09, 3 March 2022DeSlfixed title
121760view12:08, 3 March 2022DeSlCondensed PW layout
121759view12:06, 3 March 2022DeSlSmall layout changes (added one H+ DataNode)
121758view12:04, 3 March 2022DeSlConverted MIMconversion from metabolite to PW DataNode to arrows
121644view10:10, 23 February 2022SamDrabbeadjusted reference
121548view10:33, 21 February 2022SamDrabbeadded literature
121546view10:16, 21 February 2022AmauryPelzerChanged annotation (ACAT1)
121545view10:15, 21 February 2022SamDrabbechanged some line types
121542view10:11, 21 February 2022SamDrabbeOntology Term : 'CL:0000000' removed !
121541view10:11, 21 February 2022SamDrabbeOntology Term : 'native cell' added !
121539view10:10, 21 February 2022AmauryPelzerFixed an anchor point and added double directional arrows.
121538view10:08, 21 February 2022SamDrabbeOntology Term : 'Cell Type' added !
121530view08:41, 21 February 2022AmauryPelzerOntology Term : 'beta-ketothiolase deficiency' added !
121455view14:55, 17 February 2022AmauryPelzerChanged arrow types for conversions with multiple substrates/products
121454view14:52, 17 February 2022AmauryPelzerReverted to version '14:46, 17 February 2022' by AmauryPelzer

External references

DataNodes

View all...
NameTypeDatabase referenceComment
3-hydroxy-3-methylglutaryl-coenzyme AMetaboliteCHEBI:11814 (ChEBI)
3-hydroxybutyric acidMetabolite20067 (ChEBI)
ACAT1GeneProductENSG00000075239 (Ensembl) In the book by Blau et al. they used the abbreviation MAT
Acetoacetyl-CoAMetaboliteHMDB01484 (HMDB)
Acetyl-CoAMetaboliteHMDB01206 (HMDB)
BDH1ProteinC9JM78 (Uniprot-TrEMBL) In the book by Blau et al. they use the abbriviation 3HBD
Carbon DioxideMetaboliteCHEBI:16526 (ChEBI)
CoASHMetaboliteCHEBI:15346 (ChEBI)
Coenzym AMetaboliteCHEBI:15346 (ChEBI)
H+MetaboliteCHEBI:15378 (ChEBI)
HMGCLGeneProductENSG00000117305 (Ensembl)
HMGCS2ProteinP54868 (Uniprot-TrEMBL)
Leucine, isoleucine and valine metabolismPathwayWP4686 (WikiPathways)
LeucineMetaboliteCHEBI:15603 (ChEBI)
Mitochondrial long chain fatty acid beta-oxidationPathwayWP368 (WikiPathways)
NAD+MetaboliteCHEBI:15846 (ChEBI)
NADHMetaboliteCHEBI:16908 (ChEBI)
Tricarboxylic acid cyclePathwayWP78 (WikiPathways)
acetoacetateMetaboliteCHEBI:13705 (ChEBI)
acetoneMetaboliteCHEBI:15347 (ChEBI)
fatty acidsMetaboliteCHEBI:35366 (ChEBI)

Annotated Interactions

SourceTargetTypeDatabase referenceComment
3-hydroxy-3-methylglutaryl-coenzyme Amim-conversion24405 (Rhea)
Acetoacetyl-CoA3-hydroxy-3-methylglutaryl-coenzyme Amim-conversion10189 (Rhea)
Acetyl-CoAAcetoacetyl-CoAmim-conversion21039 (Rhea)
acetoacetate3-hydroxybutyric acidmim-conversion20524 (Rhea)
acetoacetateacetonemim-conversion19730 (Rhea)
Retrieved from "https://classic.wikipathways.org/index.php/Pathway:WP5175"
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