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

Gene name
HMGCL, HL
Definition
(RefSeq) 3-hydroxy-3-methylglutaryl-CoA lyase
  KO
K01640  hydroxymethylglutaryl-CoA lyase [EC:4.1.3.4]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00072  Synthesis and degradation of ketone bodies
hsa00280  Valine, leucine and isoleucine degradation
hsa00650  Butanoate metabolism
hsa01100  Metabolic pathways
hsa04146  Peroxisome
Module
hsa_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA
Network
nt06024  Valine, leucine and isoleucine degradation
  Element
N00833  HMGCL deficiency in leucine degradation
N00851  Leucine degradation
Disease
H00179  3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09101 Carbohydrate metabolism
   00650 Butanoate metabolism
    3155 (HMGCL)
  09103 Lipid metabolism
   00072 Synthesis and degradation of ketone bodies
    3155 (HMGCL)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    3155 (HMGCL)
 09140 Cellular Processes
  09141 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)
SSDB
Motif
Pfam: HMGL-like
Other DBs
NCBI-GeneID: 3155
NCBI-ProteinID: NP_000182
OMIM: 613898
HGNC: 5005
Ensembl: ENSG00000117305
Vega: OTTHUMG00000002963
Pharos: P35914(Tbio)
UniProt: P35914
Structure
PDB: 
2CW6 3MP4 3MP5 3MP3

Position
1p36.11
AA seq 325 aa
MAAMRKALPRRLVGLASLRAVSTSSMGTLPKRVKIVEVGPRDGLQNEKNIVSTPVKIKLI
DMLSEAGLSVIETTSFVSPKWVPQMGDHTEVLKGIQKFPGINYPVLTPNLKGFEAAVAAG
AKEVVIFGAASELFTKKNINCSIEESFQRFDAILKAAQSANISVRGYVSCALGCPYEGKI
SPAKVAEVTKKFYSMGCYEISLGDTIGVGTPGIMKDMLSAVMQEVPLAALAVHCHDTYGQ
ALANTLMALQMGVSVVDSSVAGLGGCPYAQGASGNLATEDLVYMLEGLGIHTGVNLQKLL
EAGNFICQALNRKTSSKVAQATCKL
NT seq 978 nt   +upstreamnt  +downstreamnt
atggcagcaatgaggaaggcgcttccgcggcgactggtgggcttggcgtccctccgggct
gtcagcacctcatctatgggcactttaccaaagcgggtgaaaattgtggaagttggtccc
cgagatggactacaaaatgaaaagaatatcgtatctactccagtgaaaatcaagctgata
gacatgctttctgaagcaggactctctgttatagaaaccaccagctttgtgtctcctaag
tgggttccccagatgggtgaccacactgaagtcttgaagggcattcagaagtttcctggc
atcaactacccagtcctgaccccaaatttgaaaggcttcgaggcagcggttgctgctgga
gccaaggaagtagtcatctttggagctgcctcagagctcttcaccaagaagaacatcaat
tgttccatagaggagagttttcagaggtttgacgcaatcctgaaggcagcgcagtcagcc
aatatttctgtgcgggggtacgtctcctgtgctcttggctgcccttatgaagggaagatc
tccccagctaaagtagctgaggtcaccaagaagttctactcaatgggctgctacgagatc
tccctgggggacaccattggtgtgggcaccccagggatcatgaaagacatgctatctgct
gtcatgcaggaagtgcctctggctgccctggctgtccactgccatgacacctatggtcaa
gccctggccaacaccttgatggccctgcagatgggagtgagtgtcgtggactcttctgtg
gcaggacttggaggctgtccctacgcacagggggcatcaggaaacttggccacagaagac
ctggtctacatgctagagggcttgggcattcacacgggtgtgaatctccagaagcttctg
gaagctggaaactttatctgtcaagccctgaacagaaaaactagctccaaagtggctcag
gctacctgtaaactctga

  All links  
Ontology (2)   
   KEGG BRITE (2)   
Pathway (7)   
   KEGG PATHWAY (5)   
   KEGG MODULE (2)   
Network (2)   
   KEGG NETWORK (2)   
Disease (3)   
   KEGG DISEASE (1)   
   OMIM (2)   
Chemical substance (6)   
   KEGG COMPOUND (6)   
Chemical reaction (3)   
   KEGG ENZYME (1)   
   KEGG REACTION (2)   
Genome (1)   
   KEGG GENOME (1)   
Gene (12)   
   KEGG ORTHOLOGY (1)   
   RefGene (2)   
   NCBI-PROTEINID (1)   
   NCBI-Gene (1)   
   HGNC (1)   
   Ensembl (1)   
   RIKEN BRC-DNA (2)   
   OC (1)   
   PHAROS (1)   
   VEGA (1)   
Protein sequence (4)   
   UniProt (1)   
   SWISS-PROT (1)   
   RefSeq(pep) (2)   
DNA sequence (32)   
   RefSeq(nuc) (2)   
   GenBank (15)   
   EMBL (15)   
Protein domain (1)   
   Pfam (1)   
All databases (73)   

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KEGG   Homo sapiens (human): 54511
Entry
54511             CDS       T01001                                 

Gene name
HMGCLL1, ERCHL, bA418P12.1, er-cHL
Definition
(RefSeq) 3-hydroxymethyl-3-methylglutaryl-CoA lyase like 1
  KO
K01640  hydroxymethylglutaryl-CoA lyase [EC:4.1.3.4]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00072  Synthesis and degradation of ketone bodies
hsa00280  Valine, leucine and isoleucine degradation
hsa00650  Butanoate metabolism
hsa01100  Metabolic pathways
hsa04146  Peroxisome
Module
hsa_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA
Network
nt06024  Valine, leucine and isoleucine degradation
  Element
N00851  Leucine degradation
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09101 Carbohydrate metabolism
   00650 Butanoate metabolism
    54511 (HMGCLL1)
  09103 Lipid metabolism
   00072 Synthesis and degradation of ketone bodies
    54511 (HMGCLL1)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    54511 (HMGCLL1)
 09140 Cellular Processes
  09141 Transport and catabolism
   04146 Peroxisome
    54511 (HMGCLL1)
Enzymes [BR:hsa01000]
 4. Lyases
  4.1  Carbon-carbon lyases
   4.1.3  Oxo-acid-lyases
    4.1.3.4  hydroxymethylglutaryl-CoA lyase
     54511 (HMGCLL1)
SSDB
Motif
Pfam: HMGL-like
Other DBs
NCBI-GeneID: 54511
NCBI-ProteinID: NP_061909
OMIM: 619050
HGNC: 21359
Ensembl: ENSG00000146151
Vega: OTTHUMG00000014902
Pharos: Q8TB92(Tbio)
UniProt: Q8TB92 O95896
Position
6p12.1
AA seq 370 aa
MGNVPSAVKHCLSYQQLLREHLWIGDSVAGALDPAQTSLLTNLHCFQPDVSGFSVSLAGT
VACIHWETSQLSGLPEFVKIVEVGPRDGLQNEKVIVPTDIKIEFINRLSQTGLSVIEVTS
FVSSRWVPQMADHTEVMKGIHQYPGVRYPVLTPNLQGFHHAVAAGATEISVFGAASESFS
KKNINCSIEESMGKFEEVVKSARHMNIPARGYVSCALGCPYEGSITPQKVTEVSKRLYGM
GCYEISLGDTIGVGTPGSMKRMLESVMKEIPPGALAVHCHDTYGQALANILTALQMGINV
VDSAVSGLGGCPYAKGASGNVATEDLIYMLNGLGLNTGVNLYKVMEAGDFICKAVNKTTN
SKVAQASFNA
NT seq 1113 nt   +upstreamnt  +downstreamnt
atggggaatgtgccatccgcggtgaagcactgcctcagctaccagcagcttctccgggag
catctctggatcggggattcagtggcaggggcgctcgaccccgcgcagacttctcttcta
acaaaccttcactgctttcagcccgatgtctctggcttctcagtctccttggcaggcacg
gtggcttgtatccactgggaaacatcccagttatctggactccctgagtttgttaaaata
gtagaagttgggcctagggatggattgcagaatgaaaaggttatagttcctacagatata
aaaattgaatttatcaatcgactttcccaaactggcttgtctgtaatagaagtgactagc
tttgtgtcttccagatgggtaccacagatggctgatcacactgaagtaatgaaaggcatt
catcaatatccaggagttcgctatcctgtccttactcctaatcttcagggttttcaccat
gctgttgctgctggagctactgagatatcagtttttggagctgcatctgaatcctttagc
aagaagaatattaactgttccattgaagaaagtatgggaaaatttgaggaggttgttaag
tctgcaagacacatgaatattccagcacgagggtatgtgtcttgtgctctgggctgtcca
tatgaaggaagtattacaccgcaaaaagtgacagaagtgtctaagagattgtacggcatg
ggttgttatgagatctctctaggagacacaattggagtgggaactccaggaagtatgaaa
agaatgttggaaagtgtgatgaaagaaatcccaccaggtgctcttgctgttcactgtcat
gacacatacggacaagccttagcaaatatccttacggcccttcagatgggaattaatgtg
gtggactccgcagtatccggattaggtggctgcccttatgcaaaaggtgcttctgggaat
gtagccactgaggatttgatatatatgcttaatggcctggggctcaatacaggtgtgaat
ctatacaaagtgatggaagctggtgactttatttgcaaagctgtgaataaaaccacaaac
tctaaagtagcacaagcctccttcaatgcttga

  All links  
Ontology (2)   
   KEGG BRITE (2)   
Pathway (7)   
   KEGG PATHWAY (5)   
   KEGG MODULE (2)   
Network (1)   
   KEGG NETWORK (1)   
Disease (1)   
   OMIM (1)   
Chemical substance (6)   
   KEGG COMPOUND (6)   
Chemical reaction (3)   
   KEGG ENZYME (1)   
   KEGG REACTION (2)   
Genome (1)   
   KEGG GENOME (1)   
Gene (11)   
   KEGG ORTHOLOGY (1)   
   RefGene (2)   
   NCBI-PROTEINID (1)   
   NCBI-Gene (1)   
   HGNC (1)   
   Ensembl (1)   
   RIKEN BRC-DNA (1)   
   OC (1)   
   PHAROS (1)   
   VEGA (1)   
Protein sequence (12)   
   UniProt (2)   
   SWISS-PROT (1)   
   RefSeq(pep) (9)   
DNA sequence (31)   
   RefSeq(nuc) (11)   
   GenBank (10)   
   EMBL (10)   
Protein domain (1)   
   Pfam (1)   
All databases (76)   

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