Disorders in ketone body synthesis (Homo sapiens)

From WikiPathways

Revision as of 12:27, 16 March 2022 by SamDrabbe (Talk | contribs)
Jump to: navigation, search
1Connection to pathwayFatty acid metabolismPyruvate metabolismSterol biosynthesis 22Cytosol 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

Please use the New WikiPathways

View approved pathways at the new wikipathways.org. This website will shutdown soon.

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
140669view19:06, 30 September 2025EgonwH⁺ with unicode
137688view05:15, 7 March 2025EgonwModified description
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 !

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-CoAMetaboliteCHEBI:15345 (ChEBI)
Acetyl-CoAMetaboliteCHEBI:57288 (ChEBI)
BDH1ProteinQ02338 (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)
Pyruvate metabolismPathway
Sterol biosynthesis PathwayWP4718 (WikiPathways)
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://oldclassic.wikipathways.org/index.php/Pathway:WP5175"
Personal tools