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Mouse Anti-HMGCL Recombinant Antibody (9F5AB9) (CBMAB-H2517-FY)

This product is mouse antibody that recognizes HMGCL. The antibody 9F5AB9 can be used for immunoassay techniques such as: IP, IF, ELISA.
See all HMGCL antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
9F5AB9
Antibody Isotype
IgG2b
Application
IP, IF, ELISA

Basic Information

Specificity
Human
Antibody Isotype
IgG2b
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Concentration
1 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
3-Hydroxymethyl-3-Methylglutaryl-CoA Lyase
Introduction
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.
Entrez Gene ID
3155
UniProt ID
P35914
Alternative Names
3-Hydroxymethyl-3-Methylglutaryl-CoA Lyase; 3-Hydroxymethyl-3-Methylglutaryl-Coenzyme A Lyase; 3-Hydroxy-3-Methylglutarate-CoA Lyase; Hydroxymethylglutaricaciduria; HMG-CoA Lyase; EC 4.1.3.
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.
Biological Process
Ketone body biosynthetic process Source: UniProtKB
Leucine catabolic process Source: GO_Central
Lipid metabolic process Source: BHF-UCL
Mitochondrion organization Source: Ensembl
Cellular Location
Mitochondrion matrix; Peroxisome. Unprocessed form is peroxisomal.
Involvement in disease
3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD):
An autosomal recessive disease affecting ketogenesis and L-leucine catabolism. The disease usually appears in the first year of life after a fasting period and its clinical acute symptoms include vomiting, seizures, metabolic acidosis, hypoketotic hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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