Gene Symbol | HMGCL |
---|---|
Entrez Gene | 3155 |
Alt Symbol | HL |
Species | Human |
Gene Type | protein-coding |
Description | 3-hydroxymethyl-3-methylglutaryl-CoA lyase |
Other Description | 3-hydroxy-3-methylglutarate-CoA lyase|3-hydroxy-3-methylglutaryl-CoA lyase|3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase|HMG-CoA lyase|hydroxymethylglutaricaciduria|hydroxymethylglutaryl-CoA lyase, mitochondrial|mitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase |
Swissprots | Q96FP8 B7UCC6 D3Y5K7 B4DUP4 P35914 Q6IBC0 |
Accessions | AER36554 EAW95094 EAW95095 EAW95096 EAW95097 P35914 AI202943 AK122801 BAG53736 AK300733 BAG62406 AK309129 AK313869 BAG36597 BC010570 AAH10570 BC021145 BM981610 BT009792 AAP88794 CA306929 CR456884 CAG33165 DA425822 DQ892083 ABM83009 DQ895276 ABM86202 FJ472654 ACK58684 GU433941 ADD21697 L07033 AAA92733 NM_000191 NP_000182 NM_001166059 NP_001159531 |
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. {ECO:0000269|PubMed:22847177, ECO:0000269|PubMed:22865860, ECO:0000269|PubMed:8566388}. |
Subcellular Location | Mitochondrion matrix. Peroxisome. Note=Unprocessed form is peroxisomal. |
Tissue Specificity | Highest expression in liver. Expressed in pancreas, kidey, intestine, testis, fibroblasts and lymphoblasts. Very low expression in brain and skeletal muscle. The relative expression of isoform 2 (at mRNA level) is highest in heart (30%), skeletal muscle (22%), and brain (14%). {ECO:0000269|PubMed:21952825}. |
Top Pathways | Peroxisome, Synthesis and degradation of ketone bodies, Valine, leucine and isoleucine degradation, Butanoate metabolism |
HMGCL/HMGCLL1 (H-50) - sc-292720 from Santa Cruz Biotechnology
|
||||||||||
HMGCL (T-20) - sc-101973 from Santa Cruz Biotechnology
|
||||||||||
HMGCL (63Z) - sc-100548 from Santa Cruz Biotechnology
|
||||||||||
HMGCL (G-12) - sc-131552 from Santa Cruz Biotechnology
|
||||||||||
HMGCL (Y-21) - sc-133661 from Santa Cruz Biotechnology
|
||||||||||
Anti-HMGCL antibody - ab104500 from Abcam
|
||||||||||
Anti-HMGCL antibody - ab55173 from Abcam
|
||||||||||
Anti-HMGCL antibody [9F5AB9] - ab110295 from Abcam
|
||||||||||
Anti-HMGCL antibody [EPR15550] - N-terminal - ab187648 from Abcam
|
||||||||||
Anti-HMGCL antibody [EPR15551] - ab197022 from Abcam
|
||||||||||
Anti-HMGCL Antibody - LS-C178400 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa1-326, clone 10D211, Azide-free) - LS-C183702 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa81-130) - LS-C40341 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (C-Terminus) - LS-C40342 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody - LS-C310625 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa160-190) - LS-C322009 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody - LS-C310628 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa70-99) - LS-C322010 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa1-325) - LS-C186043 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa160-190, AP) - LS-C258637 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa160-190, APC) - LS-C258638 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa160-190, Biotin) - LS-C258639 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa251-300) - LS-C170247 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa161-190) - LS-C164935 from LifeSpan Bioscience
|
||||||||||
Anti-HMGCL Antibody (aa71-99) - LS-C159851 from LifeSpan Bioscience
|