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

Name: Mouse Anti-HMGCL Recombinant Antibody (9F5AB9)
SKU: CBMAB-H2517-FY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

9F5AB9

Application

IP, IF, ELISA

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.

More Infomation

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.

Zhong, C., Xiong, G., Yang, H., Du, X., Du, J., Yao, F., ... & Deng, Y. (2023). Phosphorylation by IKKβ promotes the degradation of HMGCL via NEDD4 in lung cancer. International Journal of Biological Sciences, 19(4), 1110.

Cui, X., Yun, X., Sun, M., Li, R., Lyu, X., Lao, Y., ... & Yu, W. (2023). HMGCL-induced β-hydroxybutyrate production attenuates hepatocellular carcinoma via DPP4-mediated ferroptosis susceptibility. Hepatology International, 17(2), 377-392.

Li, Y., Fan, Z., Yin, D., Jiang, R., Deng, J., & Song, X. (2022). HMGCL: Heterogeneous multigraph contrastive learning for LBSN friend recommendation. World Wide Web, 1-24.

Kil, E. J. B. (2018). CRISPR-Cas9 mediated HMGCL KO in 3xTg AD mice reduces the cognitive deficit improvement seen in an intermittent metabolic switching regimen.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Antibody Pairs

HMGCL Matched Antibody Pair (560) (CAT#: APMAB-560LY)

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HMGCL Matched Antibody Pair (560) (CAT#: APMAB-560LY)

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Isotype control

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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