HMGCL Antibody (N-term)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- 产品详情
- 实验流程
- 背景知识
Application
| WB, E |
|---|---|
| Primary Accession | P35914 |
| Other Accession | NP_000182.2 |
| Reactivity | Human |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | Rabbit IgG |
| Calculated MW | 34360 Da |
| Antigen Region | 71-99 aa |
| Gene ID | 3155 |
|---|---|
| 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 71-99 amino acids from the N-terminal region of human HMGCL. |
| Dilution | WB~~1:1000 E~~Use at an assay dependent concentration. |
| 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 (N-term) is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | HMGCL |
|---|---|
| Function | Mitochondrial 3-hydroxy-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%). |
For Research Use Only. Not For Use In Diagnostic Procedures.
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
The protein encoded by this gene 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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