Protein Name: Hydroxymethylglutaryl-CoA lyase, mitochondrial
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UniprotKB/SwissProt ID: HMGCL_HUMAN (P35914)
Gene Name: HMGCL
Organism: Homo sapiens (Human).
Function: 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.
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion matrix. Peroxisome. Note=Unprocessed form is peroxisomal.
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PDB :
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Protein disease:
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| Disease database |
Database Entry |
Disease information | | HPRD | 02003 | HMG-CoA lyase deficiency |
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Network with metabolic pathway:
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Graphical Visualization of S-nitrosylation Sites:
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| Overview of Protein S-nitrosylation Sites with Functional and Structural Information |  |
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