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
Brite
KEGG Orthology (KO) [BR:hsa00001]
 Metabolism
  Carbohydrate metabolism
   00650 Butanoate metabolism
    3155 (HMGCL)
  Lipid metabolism
   00072 Synthesis and degradation of ketone bodies
    3155 (HMGCL)
  Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    3155 (HMGCL)
 Cellular Processes
  Transport and catabolism
   04146 Peroxisome
    3155 (HMGCL)
Enzymes [BR:hsa01000]
 4. Lyases
  4.1  Carbon-carbon lyases
   4.1.3  Oxo-acid-lyases
    4.1.3.4  hydroxymethylglutaryl-CoA lyase
     3155 (HMGCL)
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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