HMGCL Antibody (Center)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application
| WB, E |
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Primary Accession | P35914 |
Other Accession | Q8HXZ6, Q29448, NP_000182.2, NP_001159531.1 |
Reactivity | Human |
Predicted | Bovine, Monkey |
Host | Rabbit |
Clonality | Polyclonal |
Isotype | Rabbit IgG |
Calculated MW | 34360 Da |
Antigen Region | 161-190 aa |
Gene ID | 3155 |
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Other Names | Hydroxymethylglutaryl-CoA lyase, mitochondrial, HL, HMG-CoA lyase, 3-hydroxy-3-methylglutarate-CoA lyase, HMGCL |
Target/Specificity | This HMGCL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 161-190 amino acids from the Central region of human HMGCL. |
Dilution | WB~~1:1000 |
Format | Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
Storage | Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
Precautions | HMGCL Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures. |
Name | HMGCL |
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Function | Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3- methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. 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. |
Cellular Location | Mitochondrion matrix {ECO:0000250|UniProtKB:P38060}. Peroxisome {ECO:0000250|UniProtKB:P38060}. Note=Unprocessed form is peroxisomal {ECO:0000250|UniProtKB:P38060} |
Tissue Location | 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 (22%), and brain (14%). |
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Provided below are standard protocols that you may find useful for product applications.
Background
HMGCL 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. [provided by RefSeq].
References
Fu, Z., et al. J. Biol. Chem. 285(34):26341-26349(2010)
Pierron, S., et al. Arch Pediatr 17(1):10-13(2010)
Menao, S., et al. Hum. Mutat. 30 (3), E520-E529 (2009) :
Lin, W.D., et al. Clin. Chim. Acta 401 (1-2), 33-36 (2009) :
Carrasco, P., et al. Mol. Genet. Metab. 91(2):120-127(2007)
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