![Immunocytochemistry - HMGCL antibody [9F5AB9] (ab110295) image](#HMGCL-Primary-antibodies-ab110295-1.jpg)
Loading...
You have changed your country from
to
. Please be aware that this will change the currency in the purchasing process.
Reassurance, Refunds & Replacements
If your product does not perform as described on this datasheet, we will refund or replace your product...
Read our guarantee »Overview
Product name
Anti-HMGCL antibody [9F5AB9]
See all HMGCL products (5) ...
Description
Mouse monoclonal [9F5AB9] to HMGCL
Tested applications
ICC/IF, In-Cell ELISA, IPmore details
Cross reactivity
Reacts with
Human
Immunogen
Human liver mitochondria
Positive control
HeLa cells and human liver mitochondria.
Properties
Form
Liquid
Storage instructions
Store at +4°C. Do not freeze.
Storage buffer
Preservative: 0.02% Sodium azide
Constituent: HBS
Concentration
Concentration information loading...
Purity
Ammonium Sulphate Precipitation
Purification notes
Produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.
Clonality
Monoclonal
Clone number
9F5AB9
Isotype
IgG2b
Light chain type
kappa
Research areas
Metabolism >> Types of disease >> Cancer
Metabolism >> Pathways and Processes >> Metabolic signaling pathways >> Amino acid metabolism
Metabolism >> Pathways and Processes >> Metabolic signaling pathways >> Lipid and lipoprotein metabolism >> Lipid metabolism
Cancer >> Cancer Metabolism >> Metabolic signaling pathway >> Metabolism of lipids and lipoproteins
Signal Transduction >> Metabolism >> Lipid metabolism
Signal Transduction >> Metabolism >> Amino Acids
-
Immunocytochemistry - HMGCL antibody [9F5AB9] (ab110295)
![Immunocytochemistry - HMGCL antibody [9F5AB9] (ab110295) image](/ps/datasheet/images/110/ab110295/HMGCL-Primary-antibodies-ab110295-1.jpg)
(enlarge)
Applications
Show applications key
Our Abpromise guarantee covers the use of ab110295 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
ICC/IF: Use a concentration of 4 µg/ml.
In-Cell ELISA: Use a concentration of 1 µg/ml. (0.1 µg/well)
IP: Use at an assay dependent dilution.
Target
Function
Involved in the catabolism of branched amino acids such as leucine.
Tissue specificity
Fibroblasts, liver and lymphoblasts.
Pathway
Metabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1.
Involvement in disease
Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:246450]; 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.
Sequence similarities
Belongs to the HMG-CoA lyase family.
Cellular localization
Mitochondrion matrix.
Target information above from: UniProt accessionP35914
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- mitochondrial antibody
- 3 hydroxy 3 methylglutaryl CoA lyase antibody
- 3 hydroxy 3 methylglutaryl Coenzyme A lyase antibody
see all
Database links
- Clone ID: BC010570 Human
- Entrez Gene: 3155 Human
- IMAGE Id: 4178704 Human
- Omim: 246450 Human
- SwissProt: P35914 Human
- Unigene: 533444 Human
Anti-HMGCL antibody [9F5AB9] images:
Immunocytochemistry - HMGCL antibody [9F5AB9] (ab110295)
Immunocytochemistry image of ab110295 stained Human HeLa cells. The cells were paraformaldehyde fixed (4%, 20 minutes) and Triton X-100 permeabilized (0.1%, 15 minutes). The cells were incubated with ab110295 at 5 µg/ml for 2 hours at room temperature or over night at 4°C. The secondary antibody was (green) Alexa Fluor® 488 goat anti-mouse IgG (H+L) used at a 1/1000 dilution for 1 hour. 10% Goat serum was used as the blocking agent for all blocking steps. Target protein locates mainly in mitochondria.
References for Anti-HMGCL antibody [9F5AB9] (ab110295)
ab110295 has not yet been referenced specifically in any publications.
Publishing research using ab110295? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"