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

Scan QR to view Datasheet Catalog #    MBS7045575
Unit / Price
0.05 mL  /  $120 +1 FREE 8GB USB
0.1 mL  /  $160 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 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
 Product Gene Name   

anti-HMGCL antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    246450
 3D Structure    ModBase 3D Structure for P35914
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Rat
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 Purity/Purification    Antigen Affinity Purified
 Form/Format    Liquid
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 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
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 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.
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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)

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 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
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 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
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 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
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NCBI/Uniprot data below describe general gene information for HMGCL. It may not necessarily be applicable to this product.
 NCBI GI #    62198232
 NCBI GeneID    3155
 NCBI Accession #    NP_000182.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_000191.2 [Other Products]
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 UniProt Primary Accession #    P35914 [Other Products]
 UniProt Secondary Accession #    Q6IBC0; Q96FP8; B4DUP4; B7UCC6; D3Y5K7 [Other Products]
 UniProt Related Accession #    P35914 [Other Products]
 Molecular Weight    20,222 Da
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 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   
HL
[Similar Products]
 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
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 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]
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 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
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 Research Articles on HMGCL    1. this is the first study describing HMGCL deficiency caused by uniparental disomy.
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 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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Pathways associated with anti-HMGCL antibodyDiseases associated with anti-HMGCL antibody
 Products by Pathway  Pathway Diagram
 Butanoate Metabolism Pathway antibodies  Butanoate Metabolism Pathway Diagram
 Butanoate Metabolism Pathway antibodies  Butanoate Metabolism Pathway Diagram
 Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway antibodies  Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway Diagram
 Ketone Body Metabolism Pathway antibodies  Ketone Body Metabolism Pathway Diagram
 Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway antibodies  Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway Diagram
 Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway antibodies  Leucine Degradation, Leucine => Acetoacetate + Acetyl-CoA Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Lipids And Lipoproteins Pathway antibodies  Metabolism Of Lipids And Lipoproteins Pathway Diagram
 Peroxisome Pathway antibodies  Peroxisome Pathway Diagram
 Disease Name  Pubmed Publications
 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency Antibodies  >3 publications with HMGCL and 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
 Inflammation Antibodies  >2 publications with HMGCL and Inflammation
 Prostatic Diseases Antibodies  >1 publications with HMGCL and Prostatic Diseases
 Breast Neoplasms Antibodies  >1 publications with HMGCL and Breast Neoplasms
 Disease Models, Animal Antibodies  >1 publications with HMGCL and Disease Models, Animal
 Liver Diseases Antibodies  >1 publications with HMGCL and Liver Diseases
 Fatty Liver Antibodies  >1 publications with HMGCL and Fatty Liver
Organs/Tissues associated with anti-HMGCL antibody
 Organ/Tissue Name  Pubmed Publications
 Liver Antibodies  >5 publications with HMGCL and Liver
 Blood Antibodies  >4 publications with HMGCL and Blood
 Brain Antibodies  >4 publications with HMGCL and Brain
 Heart Antibodies  >2 publications with HMGCL and Heart
 Adipose Tissue Antibodies  >1 publications with HMGCL and Adipose Tissue
 Muscle Antibodies  >1 publications with HMGCL and Muscle
 Prostate Antibodies  >1 publications with HMGCL and Prostate
 Connective Tissue Antibodies  >1 publications with HMGCL and Connective Tissue
 Skin Antibodies  >1 publications with HMGCL and Skin
 Kidney Antibodies  >1 publications with HMGCL and Kidney
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