UniProt ID | HMGCL_HUMAN | |
---|---|---|
UniProt AC | P35914 | |
Protein Name | Hydroxymethylglutaryl-CoA lyase, mitochondrial | |
Gene Name | HMGCL | |
Organism | Homo sapiens (Human). | |
Sequence Length | 325 | |
Subcellular Localization | Mitochondrion matrix. Peroxisome. Unprocessed form is peroxisomal. | |
Protein Description | Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism.. | |
Protein Sequence | MAAMRKALPRRLVGLASLRAVSTSSMGTLPKRVKIVEVGPRDGLQNEKNIVSTPVKIKLIDMLSEAGLSVIETTSFVSPKWVPQMGDHTEVLKGIQKFPGINYPVLTPNLKGFEAAVAAGAKEVVIFGAASELFTKKNINCSIEESFQRFDAILKAAQSANISVRGYVSCALGCPYEGKISPAKVAEVTKKFYSMGCYEISLGDTIGVGTPGIMKDMLSAVMQEVPLAALAVHCHDTYGQALANTLMALQMGVSVVDSSVAGLGGCPYAQGASGNLATEDLVYMLEGLGIHTGVNLQKLLEAGNFICQALNRKTSSKVAQATCKL | |
Overview of Protein Modification Sites with Functional and Structural Information | ||
|
* ASA = Accessible Surface Area
Locations | Modification | Substrate Peptides & Secondary Structure |
ASA (%) | Reference | Orthologous Protein Cluster |
---|---|---|---|---|---|
17 | Phosphorylation | RRLVGLASLRAVSTS HHHHHHHHHHHCCCC | 24.35 | 23186163 | |
22 | Phosphorylation | LASLRAVSTSSMGTL HHHHHHCCCCCCCCC | 22.91 | 26434776 | |
23 | Phosphorylation | ASLRAVSTSSMGTLP HHHHHCCCCCCCCCC | 20.44 | 26434776 | |
48 | Succinylation | RDGLQNEKNIVSTPV CCCCCCCCCCCCCCC | 60.57 | 23954790 | |
48 | Malonylation | RDGLQNEKNIVSTPV CCCCCCCCCCCCCCC | 60.57 | 26320211 | |
48 | Ubiquitination | RDGLQNEKNIVSTPV CCCCCCCCCCCCCCC | 60.57 | 19608861 | |
48 | Acetylation | RDGLQNEKNIVSTPV CCCCCCCCCCCCCCC | 60.57 | 19608861 | |
48 | Succinylation | RDGLQNEKNIVSTPV CCCCCCCCCCCCCCC | 60.57 | - | |
52 | Phosphorylation | QNEKNIVSTPVKIKL CCCCCCCCCCCHHHH | 24.17 | 30266825 | |
53 | Phosphorylation | NEKNIVSTPVKIKLI CCCCCCCCCCHHHHH | 22.62 | 30266825 | |
78 | Phosphorylation | IETTSFVSPKWVPQM EEECCCCCCCCCCCC | 20.82 | 24275569 | |
80 | Methylation | TTSFVSPKWVPQMGD ECCCCCCCCCCCCCC | 54.00 | - | |
93 | Acetylation | GDHTEVLKGIQKFPG CCHHHHHHHHHHCCC | 60.17 | 23954790 | |
93 | Malonylation | GDHTEVLKGIQKFPG CCHHHHHHHHHHCCC | 60.17 | 26320211 | |
93 | Ubiquitination | GDHTEVLKGIQKFPG CCHHHHHHHHHHCCC | 60.17 | 19608861 | |
111 | Malonylation | PVLTPNLKGFEAAVA CEECCCCCHHHHHHH | 69.10 | 26320211 | |
111 | Acetylation | PVLTPNLKGFEAAVA CEECCCCCHHHHHHH | 69.10 | 23236377 | |
137 | Malonylation | ASELFTKKNINCSIE HHHHHHCCCCCCCHH | 61.06 | 26320211 | |
137 | Succinylation | ASELFTKKNINCSIE HHHHHHCCCCCCCHH | 61.06 | - | |
137 | Acetylation | ASELFTKKNINCSIE HHHHHHCCCCCCCHH | 61.06 | 23749302 | |
137 | Succinylation | ASELFTKKNINCSIE HHHHHHCCCCCCCHH | 61.06 | - | |
179 | Succinylation | LGCPYEGKISPAKVA CCCCCCCCCCHHHHH | 28.24 | - | |
179 | Acetylation | LGCPYEGKISPAKVA CCCCCCCCCCHHHHH | 28.24 | - | |
179 | Succinylation | LGCPYEGKISPAKVA CCCCCCCCCCHHHHH | 28.24 | - | |
181 | Phosphorylation | CPYEGKISPAKVAEV CCCCCCCCHHHHHHH | 23.45 | 24719451 | |
198 | Phosphorylation | KFYSMGCYEISLGDT HHHHCCCEEEECCCC | 15.57 | 22817900 | |
314 | Phosphorylation | CQALNRKTSSKVAQA HHHHCCCCCHHHHHH | 34.69 | 26074081 | |
315 | Phosphorylation | QALNRKTSSKVAQAT HHHCCCCCHHHHHHH | 31.06 | 26074081 | |
316 | Phosphorylation | ALNRKTSSKVAQATC HHCCCCCHHHHHHHH | 36.06 | 26074081 | |
324 | Acetylation | KVAQATCKL------ HHHHHHHCC------ | 53.53 | 25953088 | |
324 | Malonylation | KVAQATCKL------ HHHHHHHCC------ | 53.53 | 26320211 |
Modified Location | Modified Residue | Modification | Type of Upstream Proteins | Gene Name of Upstream Proteins | UniProt AC of Upstream Proteins | Sources |
---|---|---|---|---|---|---|
Oops, there are no upstream regulatory protein records of HMGCL_HUMAN !! |
Modified Location | Modified Residue | Modification | Function | Reference | ||
---|---|---|---|---|---|---|
Oops, there are no descriptions of PTM sites of HMGCL_HUMAN !! |
* Distance = the distance between SAP position and PTM sites.
Modified Location | Modification | Variant Position (Distance <= 10) |
Residue Change | SAP | Related Disease | Reference |
---|---|---|---|---|---|---|
Oops, there are no SNP-PTM records of HMGCL_HUMAN !! |
Interacting Protein | Gene Name | Interaction Type | PPI Reference | Domain-Domain Interactions |
---|---|---|---|---|
HES1_HUMAN | HES1 | physical | 21900206 | |
DNJA1_HUMAN | DNAJA1 | physical | 21900206 | |
RN126_HUMAN | RNF126 | physical | 21900206 | |
ATS10_HUMAN | ADAMTS10 | physical | 21900206 | |
AR6P1_HUMAN | ARL6IP1 | physical | 21900206 | |
PNPT1_HUMAN | PNPT1 | physical | 26186194 | |
FOLC_HUMAN | FPGS | physical | 26186194 | |
GLSK_HUMAN | GLS | physical | 26186194 | |
HEM1_HUMAN | ALAS1 | physical | 26186194 | |
ACADV_HUMAN | ACADVL | physical | 26186194 | |
MGME1_HUMAN | MGME1 | physical | 26186194 | |
FBX21_HUMAN | FBXO21 | physical | 26186194 | |
LSM12_HUMAN | LSM12 | physical | 26344197 | |
TOM40_HUMAN | TOMM40 | physical | 26344197 | |
FOLC_HUMAN | FPGS | physical | 28514442 | |
GLSK_HUMAN | GLS | physical | 28514442 | |
HEM1_HUMAN | ALAS1 | physical | 28514442 | |
ACADV_HUMAN | ACADVL | physical | 28514442 | |
MGME1_HUMAN | MGME1 | physical | 28514442 |
Kegg Disease | ||||||
---|---|---|---|---|---|---|
There are no disease associations of PTM sites. | ||||||
OMIM Disease | ||||||
246450 | 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) | |||||
Kegg Drug | ||||||
There are no disease associations of PTM sites. | ||||||
DrugBank | ||||||
There are no disease associations of PTM sites. |
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Acetylation | |
Reference | PubMed |
"Lysine acetylation targets protein complexes and co-regulates majorcellular functions."; Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.,Olsen J.V., Mann M.; Science 325:834-840(2009). Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-48, AND MASS SPECTROMETRY. |