full name
3-hydroxy-3-methylglutaryl-CoA synthase 2

synonyms
HMGCS2, ENSG00000134240

HGNC
HGNC:5008

Entrez Gene
Gene:3158

ENSEMBL
ENSG00000134240

location
chr: 1
start: 120290619
stop: 120311555
strand: -

OMIM
OMIM:600234: HMGCS2
OMIM:605911: HMG-CoA synthase-2 deficiency

transcripts
NM_001166107.1
NM_005518.3

pathways
Butanoate metabolism [NCBI:83007] [KEGG:hsa00650]
C5 isoprenoid biosynthesis, mevalonate pathway [NCBI:413387] [KEGG:hsa_M00095]
Fatty acid, triacylglycerol, and ketone body metabolism [NCBI:1270010] [REACTOME:R-HSA-535734]
Ketone body metabolism [NCBI:1270026] [REACTOME:R-HSA-74182] [PUBMED:21479626]
Metabolic pathways [NCBI:132956] [KEGG:hsa01100]
Metabolism [NCBI:1269956] [REACTOME:R-HSA-1430728]
Metabolism of lipids and lipoproteins [NCBI:1270001] [REACTOME:R-HSA-556833]
PPAR signaling pathway [NCBI:83042] [KEGG:hsa03320] [PUBMED:15673477] [PUBMED:15828221] [PUBMED:14999402] [PUBMED:15828222] [PUBMED:16601267] [PUBMED:15828220] [PUBMED:10529898] [PUBMED:15828223] [PUBMED:16476485] [PUBMED:11921433] [PUBMED:16511590] [PUBMED:16360030]
PPARA activates gene expression [NCBI:1270030] [REACTOME:R-HSA-1989781] [PUBMED:10529898] [PUBMED:18288277] [PUBMED:11330046] [PUBMED:20936127] [PUBMED:14999402]
Regulation of lipid metabolism by Peroxisome proliferator-activated receptor alpha (PPARalpha) [NCBI:1270029] [REACTOME:R-HSA-400206] [PUBMED:18288277] [PUBMED:10529898] [PUBMED:16476485] [PUBMED:16503871]
Synthesis and degradation of ketone bodies [NCBI:82936] [KEGG:hsa00072]
Synthesis of Ketone Bodies [NCBI:1270027] [REACTOME:R-HSA-77111] [PUBMED:21479626]
Terpenoid backbone biosynthesis [NCBI:83022] [KEGG:hsa00900] [PUBMED:12777052] [PUBMED:9858571] [PUBMED:16262699] [PUBMED:24375100]
Validated targets of C-MYC transcriptional repression [NCBI:169353] [Pathway Interaction Database:myc_represspathway] [PUBMED:17082179] [PUBMED:11282029] [PUBMED:9869632] [PUBMED:15021909] [PUBMED:16167342] [PUBMED:18056446] [PUBMED:18045875] [PUBMED:1670896] [PUBMED:18393360] [PUBMED:7556187] [PUBMED:11313917] [PUBMED:17471507] [PUBMED:12418961] [PUBMED:9927431] [PUBMED:9924025] [PUBMED:18483244] [PUBMED:14716294] [PUBMED:15506972] [PUBMED:15616584] [PUBMED:12729735] [PUBMED:19219026] [PUBMED:12747840] [PUBMED:2182320] [PUBMED:12545156] [PUBMED:15367674] [PUBMED:11283613] [PUBMED:12384701] [PUBMED:18066065] [PUBMED:18455888] [PUBMED:10920185] [PUBMED:17881732] [PUBMED:20190815] [PUBMED:16352593] [PUBMED:17050536] [PUBMED:11283614] [PUBMED:16391002] [PUBMED:11804592] [PUBMED:7870572] [PUBMED:16940161] [PUBMED:12695333] [PUBMED:17159920] [PUBMED:8972190] [PUBMED:9312026] [PUBMED:17485441] [PUBMED:16288014]
Valine, leucine and isoleucine degradation [NCBI:82952] [KEGG:hsa00280]
ketogenesis [NCBI:545368] [BIOCYC:HUMAN_PWY66-367] [PUBMED:12364542] [PUBMED:9727714] [PUBMED:14769483] [PUBMED:10031425] [PUBMED:18668440] [PUBMED:1196305] [PUBMED:8027038] [PUBMED:17371050] [PUBMED:20491249]
mevalonate pathway [NCBI:142207] [BIOCYC:HUMAN_PWY-922]
superpathway of cholesterol biosynthesis [NCBI:142269] [BIOCYC:HUMAN_PWY66-5] [PUBMED:240818] [PUBMED:11519011] [PUBMED:9634533] [PUBMED:10666321] [PUBMED:14766201] [PUBMED:9465114] [PUBMED:10710235] [PUBMED:9683613] [PUBMED:12133002] [PUBMED:9894009] [PUBMED:12618959] [PUBMED:3037232] [PUBMED:2614264] [PUBMED:9630650] [PUBMED:10391219] [PUBMED:12829805] [PUBMED:2007571] [PUBMED:9653161]
superpathway of geranylgeranyldiphosphate biosynthesis I (via mevalonate) [NCBI:545288] [BIOCYC:HUMAN_PWY-5910]