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Products:Signal Transduction >> Metabolism >> Energy Metabolism
Overview
Product name
Anti-FH antibody
See all FH products (7) ...
Description
Mouse monoclonal to FH
Tested applications
Cross reactivity
Reacts with
Human
Immunogen
Recombinant full length protein, corresponding to amino acids 33-511 of Human FH
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage buffer
Preservative: None
PBS, pH 7.2
Concentration
Concentration information loading...
Purity
Protein G purified
Clonality
Monoclonal
Isotype
IgG2a
Light chain type
kappa
Research areas
Cancer >> Cancer Metabolism >> Metabolic signaling pathway >> Integration of energy metabolism
Cancer >> Oncoproteins/suppressors >> Tumor suppressors >> Other
Signal Transduction >> Metabolism >> Energy Metabolism
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Western blot - FH antibody (ab58232)
(enlarge)
Applications
Show applications key
Our Abpromise guarantee covers the use of ab58232 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
- 1 Image
WB
Application notes
WB: Use at a concentration of 1-5 µg/ml.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
Target
Function
Also acts as a tumor suppressor.
Pathway
Carbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1.
Involvement in disease
Defects 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 similarities
Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
Cellular localization
Cytoplasm and Mitochondrion.
Target information above from: UniProt accessionP07954
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- FH antibody
- Fumarase antibody
- Fumarate hydratase mitochondrial antibody
see all
Database links
- Entrez Gene: 2271 Human
- Omim: 136850 Human
- SwissProt: P07954 Human
- Unigene: 592490 Human
Anti-FH antibody images:
Western blot - FH antibody (ab58232)
FH antibody (ab58232) at 1ug/lane + HeLa cell lysate at 25ug/lane.
References for Anti-FH antibody (ab58232)
This product has been referenced in:
- Yang Yet al. UOK 262 cell line, fumarate hydratase deficient (FH-/FH-) hereditary leiomyomatosis renal cell carcinoma: in vitro and in vivo model of an aberrant energy metabolic pathway in human cancer. Cancer Genet Cytogenet 196:45-55 (2010).Read more (PubMed: 19963135) »
- Kienhöfer Jet al. Association of mitochondrial antioxidant enzymes with mitochondrial DNA as integral nucleoid constituents. FASEB J : (2009).Read more (PubMed: 19228881) »
See all 2 publications for this product
Publishing research using ab58232? 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"