Recombinant Human Hydroxymethylglutaryl-CoA lyase, mitochondrial(HMGCL)

Product Type Recombinant Protein
Uniprot No. P35914
Species Homo sapiens (Human)
Gene Names HMGCL
Protein Names Recommended name: Hydroxymethylglutaryl-CoA lyase, mitochondrial Short name= HL Short name= HMG-CoA lyase EC= 4.1.3.4 Alternative name(s): 3-hydroxy-3-methylglutarate-CoA lyase
Storage Store at -20°C, for extended storage, conserve at -20°C or -80°C.
Notes Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Target Details This protein belongs to the HMG-CoA lyase family. It is a mitochondrial enzyme that catalyzes the final step of leucine degradation and plays a key role in ketone body formation. Mutations in this gene are associated with HMG-CoA lyase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
HGNC 5005
RGD 620554
MGI 96158
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.
Involvement in disease 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD)
Subcellular Location Mitochondrion matrix, Peroxisome
Protein Families HMG-CoA lyase family
Tissue Specificity Highest expression in liver. Expressed in pancreas, kidney, 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
Database Links

HGNC: 5005

OMIM: 246450

KEGG: hsa:3155

STRING: 9606.ENSP00000363614

UniGene: Hs.533444

Product Details for Different Expression Systems

Product Name Recombinant Human Hydroxymethylglutaryl-CoA lyase, mitochondrial(HMGCL),Yeast
Code CSB-YP010563HU
Size Pls inquiry other sizes
Source Yeast
Purity >85% (SDS-PAGE)
Tag Info The following tags are available.
N-terminal His-tagged
Tag-Free
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Sequence TLP KRVKIVEVGP RDGLQNEKNI VSTPVKIKLI DMLSEAGLSV IETTSFVSPK WVPQMGDHTE VLKGIQKFPG INYPVLTPNL KGFEAAVAAG AKEVVIFGAA SELFTKKNIN CSIEESFQRF DAILKAAQSA NISVRGYVSC ALGCPYEGKI SPAKVAEVTK KFYSMGCYEI SLGDTIGVGT PGIMKDMLSA VMQEVPLAAL AVHCHDTYGQ ALANTLMALQ MGVSVVDSSV AGLGGCPYAQ GASGNLATED LVYMLEGLGI HTGVNLQKLL EAGNFICQAL NRKTSSKVAQ ATCKL
Expression Region 28-325
Protein Description Full Length of Mature Protein
Storage Buffer Lyophilized from Tris/PBS-based buffer, 6% Trehalose, pH 8.0
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Product Name Recombinant Human Hydroxymethylglutaryl-CoA lyase, mitochondrial(HMGCL),Baculovirus
Code CSB-BP010563HU
Size Pls inquiry other sizes
Source Baculovirus
Purity >85% (SDS-PAGE)
Tag Info The following tags are available.
N-terminal His-tagged and C-terminal Myc-tagged
N-terminal His-tagged
Tag-Free
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Sequence TLP KRVKIVEVGP RDGLQNEKNI VSTPVKIKLI DMLSEAGLSV IETTSFVSPK WVPQMGDHTE VLKGIQKFPG INYPVLTPNL KGFEAAVAAG AKEVVIFGAA SELFTKKNIN CSIEESFQRF DAILKAAQSA NISVRGYVSC ALGCPYEGKI SPAKVAEVTK KFYSMGCYEI SLGDTIGVGT PGIMKDMLSA VMQEVPLAAL AVHCHDTYGQ ALANTLMALQ MGVSVVDSSV AGLGGCPYAQ GASGNLATED LVYMLEGLGI HTGVNLQKLL EAGNFICQAL NRKTSSKVAQ ATCKL
Expression Region 28-325
Protein Description Full Length of Mature Protein
Storage Buffer Lyophilized from Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Still Have Questions? Leave a Message or Start an on-line Chat
Product Name Recombinant Human Hydroxymethylglutaryl-CoA lyase, mitochondrial(HMGCL),Mammalian cell
Code CSB-MP010563HU
Size Pls inquiry other sizes
Source Mammalian cell
Purity >85% (SDS-PAGE)
Tag Info The following tags are available.
N-terminal His-tagged and C-terminal Myc-tagged
N-terminal His-tagged
Tag-Free
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Sequence TLP KRVKIVEVGP RDGLQNEKNI VSTPVKIKLI DMLSEAGLSV IETTSFVSPK WVPQMGDHTE VLKGIQKFPG INYPVLTPNL KGFEAAVAAG AKEVVIFGAA SELFTKKNIN CSIEESFQRF DAILKAAQSA NISVRGYVSC ALGCPYEGKI SPAKVAEVTK KFYSMGCYEI SLGDTIGVGT PGIMKDMLSA VMQEVPLAAL AVHCHDTYGQ ALANTLMALQ MGVSVVDSSV AGLGGCPYAQ GASGNLATED LVYMLEGLGI HTGVNLQKLL EAGNFICQAL NRKTSSKVAQ ATCKL
Expression Region 28-325
Protein Description Full Length of Mature Protein
Storage Buffer Lyophilized from Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Still Have Questions? Leave a Message or Start an on-line Chat

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