Product nameAnti-FH/Fumarase antibody [EPR11648] - BSA and Azide free
See all FH/Fumarase primary antibodies
DescriptionRabbit monoclonal [EPR11648] to FH/Fumarase - BSA and Azide free
Tested applicationsSuitable for: WB, ICC/IFmore details
Unsuitable for: Flow Cyt,IHC-P or IP
Species reactivityReacts with: Mouse, Rat, Human
Synthetic peptide within Human FH/Fumarase aa 450 to the C-terminus. The exact sequence is proprietary.
Database link: P07954
ab249667 is the carrier-free version of ab171948 This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.
Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.
Use our conjugation kits for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.
Ab249667 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.
Maxpar® is a trademark of Fluidigm Canada Inc.
This product was previously labelled as FH
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferConstituent: PBS
Concentration information loading...
- Pathways and Processes
- Metabolic signaling pathways
- Energy transfer pathways
- Energy Metabolism
Our Abpromise guarantee covers the use of ab249667 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use at an assay dependent concentration. Predicted molecular weight: 50 kDa.|
|ICC/IF||Use at an assay dependent concentration.|
FunctionAlso acts as a tumor suppressor.
PathwayCarbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1.
Involvement in diseaseDefects in FH are the cause of fumarase deficiency (FHD) [MIM:606812]; also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia.
Defects in FH are the cause of multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800]. MCUL1 is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids).
Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].
Sequence similaritiesBelongs to the class-II fumarase/aspartase family. Fumarase subfamily.
Cellular localizationCytoplasm and Mitochondrion.
- Information by UniProt
- FH antibody
- Fumarase antibody
- Fumarate hydratase antibody
ab249667 has not yet been referenced specifically in any publications.