HMGCL Human

3-Hydroxymethyl-3-Methylglutaryl-CoA Lyase Human Recombinant
Cat. No.
BT6856
Source
Escherichia Coli.
Synonyms
Hydroxymethylglutaryl-CoA lyase mitochondrial, HL, HMG-CoA lyase, 3-hydroxy-3-methylglutarate-CoA lyase, HMGCL.
Appearance
Sterile Filtered colorless solution.
Purity
Greater than 85.0% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

HMGCL Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 323 amino acids (28-325) and having a molecular mass of 34.2kDa.
HMGCL is fused to a 25 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction
Hydroxymethylglutaryl-CoA lyase (HMGCL) is a mitochondrial matrix protein belonging to the HMG-CoA lyase family. This homodimeric enzyme plays a crucial role in leucine catabolism and ketogenesis, the process by which the liver synthesizes ketone bodies. During periods of fasting, ketone bodies serve as a vital energy source for organs like the heart, brain, and kidneys. HMGCL specifically catalyzes the final step of these metabolic pathways, cleaving 3-hydroxy-3-methylglutaryl-CoA into acetoacetic acid and acetyl-CoA.
Description
Recombinant human HMGCL, produced in E. coli, is a single, non-glycosylated polypeptide chain comprising 323 amino acids (residues 28-325) with a molecular weight of 34.2 kDa. This protein is expressed with a 25 amino acid His-tag fused at the N-terminus and purified using proprietary chromatographic techniques.
Physical Appearance
Sterile, colorless, and filtered solution.
Formulation
The HMGCL solution is provided at a concentration of 1 mg/ml in a buffer consisting of 20 mM Tris-HCl (pH 8.0), 10% glycerol, and 0.4 M Urea.
Stability
For short-term storage (up to 2-4 weeks), the product can be stored at 4°C. For extended storage, it is recommended to freeze the product at -20°C. To ensure long-term stability during frozen storage, adding a carrier protein such as 0.1% HSA or BSA is advised. Repeated freezing and thawing cycles should be avoided.
Purity
The purity of the HMGCL protein is greater than 85%, as determined by SDS-PAGE analysis.
Synonyms
Hydroxymethylglutaryl-CoA lyase mitochondrial, HL, HMG-CoA lyase, 3-hydroxy-3-methylglutarate-CoA lyase, HMGCL.
Source
Escherichia Coli.
Amino Acid Sequence
MGSSHHHHHH SSGLVPRGSH MGSHMTLPKR VKIVEVGPRD GLQNEKNIVS TPVKIKLIDM LSEAGLSVIE TTSFVSPKWV PQMGDHTEVL KGIQKFPGIN YPVLTPNLKG FEAAVAAGAK EVVIFGAASE LFTKKNINCS IEESFQRFDA ILKAAQSANI SVRGYVSCAL GCPYEGKISP AKVAEVTKKF YSMGCYEISL GDTIGVGTPG IMKDMLSAVM QEVPLAALAV HCHDTYGQAL ANTLMALQMG VSVVDSSVAG LGGCPYAQGA SGNLATEDLV YMLEGLGIHT GVNLQKLLEA GNFICQALNR KTSSKVAQAT CKL.

Product Science Overview

Structure and Function

HMG-CoA Lyase is a mitochondrial enzyme that catalyzes the cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate . This reaction is a key step in the formation of ketone bodies, which are essential as an alternative energy source to glucose, especially during periods of fasting or strenuous exercise . The enzyme also plays a significant role in the final step of leucine degradation .

The HMGCL gene encodes a protein that is approximately 34.5 kDa in size and is localized to both the mitochondrion and peroxisome . Multiple isoforms of the protein exist due to alternative splicing, with the major isoform being highly expressed in the liver . The structure of HMG-CoA Lyase has been resolved by X-ray crystallography, revealing that the protein functions as a dimer .

Clinical Significance

Mutations in the HMGCL gene can lead to HMG-CoA Lyase deficiency, a rare metabolic disorder characterized by hypoketotic hypoglycemia and metabolic acidosis . This condition results from the body’s inability to properly break down leucine and produce ketone bodies, leading to an accumulation of organic acids .

Recombinant HMG-CoA Lyase

Human recombinant HMG-CoA Lyase is produced using recombinant DNA technology, which involves inserting the HMGCL gene into a suitable expression system, such as bacteria or yeast, to produce the enzyme in large quantities. This recombinant enzyme is used in research to study its structure, function, and role in metabolic disorders.

Applications in Research

Recombinant HMG-CoA Lyase is valuable in biochemical research for several reasons:

  • Structural Studies: Understanding the enzyme’s structure helps in elucidating its function and the impact of mutations.
  • Metabolic Pathways: Studying the enzyme’s role in ketogenesis and leucine catabolism provides insights into metabolic diseases.
  • Drug Development: Targeting HMG-CoA Lyase can lead to potential therapies for metabolic disorders.

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