Anti-HLCS Recombinant Antibody Products

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For Research Use Only. Not For Clinical Use.


Creative Biolabs is at the forefront of rare metabolic disease research, providing top-tier anti-HLCS recombinant antibodies. As the enzyme that attaches biotin to all five human carboxylases, Holocarboxylase Synthetase (HLCS) is completely indispensable for metabolism. Our high-quality antibodies can be used to help diagnose holocarboxylase synthetase deficiency as well as to better understand the underlying biology of biotin-dependent metabolism.

HLCS: The Master Enzyme for Biotin Attachment

Holocarboxylase Synthetase (HLCS) is a unique and essential enzyme, serving as a highly specific biotin ligase. It covalently attaches the vitamin biotin to a unique lysine residue on all five biotin-dependent carboxylase enzymes in humans. These include propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, pyruvate carboxylase, 3-methylcrotonyl-CoA carboxylase, and beta-methylcrotonyl-CoA carboxylase, which are involved in fatty acid synthesis, amino acid catabolism, and gluconeogenesis. Without this modification, these carboxylases are inactive apoenzymes. Genetic deficiency of HLCS results in the failure to activate all five carboxylases, leading to a severe, neonatal-onset inborn error of metabolism, holocarboxylase synthetase deficiency, which is a form of multiple carboxylase deficiency.

Alternative Names

HLCS; Biotin--Protein Ligase; Biotin Apo-Protein Ligase; Holocarboxylase Synthetase (Biotin-(Proprionyl-CoA-Carboxylase (ATP-Hydrolysing)) Ligase); EC 6.3.4.-; Holocarboxylase Synthetase (Biotin-[Proprionyl-Coenzyme A-Carboxylase (ATP-Hydrolysing)] Ligase

Background

This gene encodes an enzyme that catalyzes the binding of biotin to carboxylases and histones. The protein is important for gluconeogenesis, fatty acid synthesis and branched chain amino acid catabolism. Defects in this gene cause holocarboxylase synthetase deficiency. Multiple alternatively spliced variants, encoding the same protein, have been identified.

Protein class

Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets

Predicted location

Intracellular

Single cell type specificity

Cell type enhanced (Inhibitory neurons, Excitatory neurons, Oligodendrocytes, Oligodendrocyte precursor cells)

Immune cell specificity

Low immune cell specificity

Cell line specificity

Low cell line specificity

Interaction

Homodimer.

Molecular function

Ligase, Multifunctional enzyme

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Anti-HLCS rAb Products

Our anti-HLCS recombinant antibodies are engineered for exceptional specificity and performance. Carefully validated to specifically detect the HLCS enzyme in cell lysates, these antibodies are optimized for applications like Western Blot to confirm the absence of protein in patient cells. Experience the reliability and precision of these rAbs for the diagnosis and study of this rare metabolic disorder.

Table 1. Featured anti-HLCS recombinant antibody products at Creative Biolabs.

Cat. No. Product Name Target Species Host Species Applications
MOB-2416z Mouse Anti-HLCS Recombinant Antibody (clone 3F7) Human Mouse IgG1 WB, FC, IHC
MOR-1627 Rabbit Anti-HLCS Recombinant Antibody (clone DS1627AB) Human Rabbit IgG WB, IHC, FC

Customer Reviews

Excellent
Da*l
Mouse Anti-HLCS Recombinant Antibody (clone 3F7)
I am always impressed with the quality of Creative Biolabs' antibodies. This anti-HLCS antibody has performed perfectly and reproducibly, which is critical for our studies on inborn errors of metabolism.
11/Jul/2024

rAb Production

For the specialized needs of metabolic research, Creative Biolabs has developed proprietary platforms for antibody production. Our anti-HLCS recombinant antibodies are expressed in an optimized system, designed to ensure they specifically recognize the native form of the enzyme. The result is a highly specific and active antibody for your HLCS experiments.

Featured Anti-HLCS Recombinant Antibody Production PlatformsFig.1 Milligram-scale. (Creative Biolabs Original)
Fig.1 Milligram-scale anti-HLCS recombinant antibody production.

Fig.2 Gram-scale. (Creative Biolabs Original)Fig.2 Gram-scale anti-HLCS recombinant antibody production.

rAb Modalities

Creative Biolabs is committed to empowering discovery by providing researchers with the most flexible antibody solutions. Our anti-HLCS recombinant antibodies are available in a variety of customizable modalities to meet the needs of your specific experimental goals, helping researchers around the world study this important enzyme.

Fig.3 HLCS antibody production and modalities. (Creative Biolabs Original)Fig.3 Full-length anti-HLCS recombinant antibody production and modalities.

Investigate the critical roles of HLCS in biotin metabolism using the highest quality recombinant antibodies from Creative Biolabs. Characterized by high specificity and strict validation standards, our portfolio contains the perfect tools for your research on rare genetic diseases. Discover quality you can trust to power discovery. Speak to our experts today.

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