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anti-HMGCL antibody :: Rabbit anti-Human, Rat HMGCL Polyclonal Antibody

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Catalog # MBS7045575
Unit / Price
  0.05 mL  /  $120 +1 FREE 8GB USB
  0.1 mL  /  $160 +1 FREE 8GB USB
Product Name

HMGCL, Polyclonal Antibody

Popular Item
Also Known As

HMGCL Antibody

Product Synonym Names
Hydroxymethylglutaryl-CoA lyase, mitochondrial; HL; HMG-CoA lyase; 3-hydroxy-3-methylglutarate-CoA lyase; HMGCL
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence Length
325
OMIM
246450
3D Structure
ModBase 3D Structure for P35914
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Rat
Purity/Purification
Antigen Affinity Purified
Form/Format
Liquid
Species
Human
Immunogen
Recombinant human Hydroxymethylglutaryl-CoA lyase, mitochondrial protein (1-325AA)
Conjugate
Non-conjugated
Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-101973 / sc-133661
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
ISO Certification
Manufactured in an ISO 9001:2008 Certified Laboratory.
Other Notes
Small volumes of anti-HMGCL antibody 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.
Related Product Information for
anti-HMGCL antibody
Key enzyme in ketogenesis (ketone body formation). 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.
Applications Tested/Suitable for anti-HMGCL antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

Western Blot (WB) of anti-HMGCL antibody
Western blot
All lanes: HMGCL antibody at 0.8ug/ml+rat liver tissue
Secondary
Goat polyclonal to rabbit at 1/10000 dilution
Predicted band size: 35,27,21 kDa
Observed band size: 34 kDa

anti-HMGCL antibody Western Blot (WB) (WB) image
Immunohistochemistry (IHC) of anti-HMGCL antibody
Immunohistochemistry of paraffin-embedded human testis tissue using MBS7045575 at dilution 1:100
anti-HMGCL antibody Immunohistochemistry (IHC) (IHC) image
Immunohistochemistry (IHC) of anti-HMGCL antibody
Immunohistochemistry of paraffin-embedded human liver tissue using MBS7045575 at dilution 1:100
anti-HMGCL antibody Immunohistochemistry (IHC) (IHC) image
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
20,222 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
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: Lyase; EC 4.1.3.4; Lipid Metabolism - synthesis and degradation of ketone bodies; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - butanoate

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

Cellular Component: mitochondrial matrix; mitochondrion; peroxisome

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

Biological Process: ketone body biosynthetic process; protein tetramerization

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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