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Mouse Anti-HMGCL Recombinant Antibody (4F4-D1) (CBMAB-A3974-LY)

The product is antibody recognizes HMGCL. The antibody 4F4-D1 immunoassay techniques such as: WB, ELISA.
See all HMGCL antibodies
Published Data

Summary

Host Animal
Mouse
Specificity
Human
Clone
4F4-D1
Antibody Isotype
IgG2a, κ
Application
WB, ELISA

Basic Information

Immunogen
HMGCL (AAH10570, 1 a.a. ~ 325 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Specificity
Human
Antibody Isotype
IgG2a, κ
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
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
3-Hydroxymethyl-3-Methylglutaryl-CoA Lyase
Entrez Gene ID
UniProt ID
Alternative Names
HL
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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