| 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 | ||
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* 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. | |