KEGG   Homo sapiens (human): 3155Help
Entry
3155              CDS       T01001                                 

Gene name
HMGCL, HL
Definition
3-hydroxymethyl-3-methylglutaryl-CoA lyase (EC:4.1.3.4)
Orthology
K01640  
hydroxymethylglutaryl-CoA lyase [EC:4.1.3.4]
Organism
hsa  Homo sapiens (human)
Pathway
Synthesis and degradation of ketone bodies
Valine, leucine and isoleucine degradation
Butanoate metabolism
Metabolic pathways
Peroxisome
Module
Leucine degradation, leucine => acetoacetate + acetyl-CoA
Disease
H00179  
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
Class
Metabolism; Carbohydrate metabolism; Butanoate metabolism [PATH:hsa00650]
Metabolism; Lipid metabolism; Synthesis and degradation of ketone bodies [PATH:hsa00072]
Metabolism; Amino acid metabolism; Valine, leucine and isoleucine degradation [PATH:hsa00280]
Cellular Processes; Transport and catabolism; Peroxisome [PATH:hsa04146]
BRITE hierarchy
SSDB OrthologParalogGFIT
Motif
Pfam: 
Motif
Other DBs
NCBI-GI: 
NCBI-GeneID: 
OMIM: 
HGNC: 
HPRD: 
Ensembl: 
Vega: 
UniProt: 
Structure
PDB: 

Jmol
Position
1p36.1-p35
AA seq 325 aa AA seqDB search
MAAMRKALPRRLVGLASLRAVSTSSMGTLPKRVKIVEVGPRDGLQNEKNIVSTPVKIKLI
DMLSEAGLSVIETTSFVSPKWVPQMGDHTEVLKGIQKFPGINYPVLTPNLKGFEAAVAAG
AKEVVIFGAASELFTKKNINCSIEESFQRFDAILKAAQSANISVRGYVSCALGCPYEGKI
SPAKVAEVTKKFYSMGCYEISLGDTIGVGTPGIMKDMLSAVMQEVPLAALAVHCHDTYGQ
ALANTLMALQMGVSVVDSSVAGLGGCPYAQGASGNLATEDLVYMLEGLGIHTGVNLQKLL
EAGNFICQALNRKTSSKVAQATCKL
NT seq 978 nt NT seq  +upstreamnt  +downstreamnt
atggcagcaatgaggaaggcgcttccgcggcgactggtgggcttggcgtccctccgggct
gtcagcacctcatctatgggcactttaccaaagcgggtgaaaattgtggaagttggtccc
cgagatggactacaaaatgaaaagaatatcgtatctactccagtgaaaatcaagctgata
gacatgctttctgaagcaggactctctgttatagaaaccaccagctttgtgtctcctaag
tgggttccccagatgggtgaccacactgaagtcttgaagggcattcagaagtttcctggc
atcaactacccagtcctgaccccaaatttgaaaggcttcgaggcagcggttgctgctgga
gccaaggaagtagtcatctttggagctgcctcagagctcttcaccaagaagaacatcaat
tgttccatagaggagagttttcagaggtttgacgcaatcctgaaggcagcgcagtcagcc
aatatttctgtgcgggggtacgtctcctgtgctcttggctgcccttatgaagggaagatc
tccccagctaaagtagctgaggtcaccaagaagttctactcaatgggctgctacgagatc
tccctgggggacaccattggtgtgggcaccccagggatcatgaaagacatgctatctgct
gtcatgcaggaagtgcctctggctgccctggctgtccactgccatgacacctatggtcaa
gccctggccaacaccttgatggccctgcagatgggagtgagtgtcgtggactcttctgtg
gcaggacttggaggctgtccctacgcacagggggcatcaggaaacttggccacagaagac
ctggtctacatgctagagggcttgggcattcacacgggtgtgaatctccagaagcttctg
gaagctggaaactttatctgtcaagccctgaacagaaaaactagctccaaagtggctcag
gctacctgtaaactctga

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