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HMGCL elisa kit :: Human 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase ELISA Kit

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Catalog # MBS9333082
Unit / Price
  48-Strip-Wells  /  $470 +1 FREE 8GB USB
  96-Strip-Wells  /  $680 +1 FREE 8GB USB
  5x96-Strip-Wells  /  $3,100 +3 FREE 8GB USB
  10x96-Strip-Wells  /  $6,095 +7 FREE 8GB USB
HMGCL elisa kit
Product Name

3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (HMGCL), ELISA Kit

Full Product Name

Human Hydroxymethylglutaryl-CoA lyase, mitochondrial, HMGCL ELISA Kit

Product Synonym Names
Human Hydroxymethylglutaryl-CoA lyase; mitochondrial (HMGCL) ELISA kit; HL; 3-hydroxy-3-methylglutaryl CoA lyase; 3-hydroxy-3-methylglutaryl-CoA lyase; HMG-CoA lyase; hydroxymethylglutaricaciduria; 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase
Product Gene Name
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Request for Current Manual Insert
OMIM
613898
3D Structure
ModBase 3D Structure for P35914
Species Reactivity
Preparation and Storage
Store all reagents at 2-8 degree C
Product Note
Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.
Other Notes
Small volumes of HMGCL elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Searchable Terms forHMGCLpurchase
MBS9333082 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (HMGCL) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing HMGCL. The ELISA analytical biochemical technique of the MBS9333082 kit is based on HMGCL antibody-HMGCL antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect HMGCL antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, HMGCL. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).
NCBI/Uniprot data below describe general gene information for HMGCL. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
34,360 Da
NCBI Official Full Name
hydroxymethylglutaryl-CoA lyase, mitochondrial isoform 1
NCBI Official Synonym Full Names
3-hydroxymethyl-3-methylglutaryl-CoA lyase
NCBI Official Symbol
HMGCL  [Similar Products]
NCBI Official Synonym Symbols
NCBI Protein Information
hydroxymethylglutaryl-CoA lyase, mitochondrial; HMG-CoA lyase; hydroxymethylglutaricaciduria; 3-hydroxy-3-methylglutaryl-CoA lyase; 3-hydroxy-3-methylglutarate-CoA lyase; 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase; mitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase
UniProt Protein Name
Hydroxymethylglutaryl-CoA lyase, mitochondrial
UniProt Synonym Protein Names
3-hydroxy-3-methylglutarate-CoA lyase
UniProt Gene Name
HMGCL  [Similar Products]
UniProt Synonym Gene Names
HL; HMG-CoA lyase  [Similar Products]
UniProt Entry Name
HMGCL_HUMAN
NCBI Summary for HMGCL
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. [provided by RefSeq, Oct 2009]
UniProt Comments for HMGCL
HMGCL: Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Defects in HMGCL are the cause of 3-hydroxy-3- methylglutaryl-CoA lyase deficiency (HMGCLD); also known as hydroxymethylglutaricaciduria or HL deficiency. 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. Belongs to the HMG-CoA lyase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Carbohydrate Metabolism - butanoate; Lipid Metabolism - synthesis and degradation of ketone bodies; Mitochondrial; EC 4.1.3.4; Lyase; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Chromosomal Location of Human Ortholog: 1p36.1-p35

Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane; peroxisome

Molecular Function: protein homodimerization activity; hydroxymethylglutaryl-CoA lyase activity; acyl-CoA binding; carboxylic acid binding; metal ion binding; manganese ion binding; magnesium ion binding; receptor binding

Biological Process: response to starvation; mitochondrion organization and biogenesis; leucine catabolic process; acyl-CoA metabolic process; ketone body biosynthetic process; cellular lipid metabolic process; ketone body metabolic process; liver development; protein tetramerization; response to nutrient

Disease: 3-hydroxy-3-methylglutaryl-coa Lyase Deficiency
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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